Skip to main content
SpringerLink
Log in

Botulinum Toxin Treatment of Primary Dystonia

  • Chapter
  • First Online:
Botulinum Toxin Treatment in Clinical Medicine

  • 1027 Accesses

Abstract

Dystonia is a sustained or intermittent muscle contraction causing abnormal movements and/or postures. There are both inherited and acquired causes of dystonia. The pathophysiology of this disorder is complex, but is known to involve circuits in the basal ganglia and cerebellum. Botulinum toxin (BoNT) is the treatment of choice for focal dystonia—including cervical dystonia, blepharospasm, and focal hand dystonia—and is useful for selected areas in generalized dystonia as well. Use of BoNT is supported by multiple studies and its safety and efficacy have been demonstrated repeatedly. Use of BoNT is limited by both dosing and by the occurrence of side effects which are due to spread of the toxin into adjacent muscles. The type of side effect(s) depends on the areas injected, but can include ptosis, diplopia, hematoma at the injection site, neck weakness, and dysphagia.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  1. Albanese A, Bhatia K, Bressman SB, et al. Phenomenology and classification of dystonia: a consensus update. Mov Disord. 2013;28:863–73.

    Article  PubMed  PubMed Central  Google Scholar 

  2. 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;13:433–44.

    Article  CAS  PubMed  Google Scholar 

  3. Balash Y, Giladi N. Efficacy of pharmacological treatment of dystonia: evidence-based review including meta-analysis of the effect of botulinum toxin and other cure options. Eur J Neurol. 2004;11:361–70.

    Article  CAS  PubMed  Google Scholar 

  4. Nutt JG, Muenter MD, Aronson A, Kurland LT, Melton LJ III. Epidemiology of focal and generalized dystonia in Rochester, Minnesota. Mov Disord. 1988;3:188–94.

    Article  CAS  PubMed  Google Scholar 

  5. Bressman SB. Dystonia update. Clin Neuropharmacol. 2000;23:239–51.

    Article  CAS  PubMed  Google Scholar 

  6. Defazio G, Jankovic J, Giel JL, Papapetropoulos S. Descriptive epidemiology of cervical dystonia. Tremor Other Hyperkinet Mov (N Y). 2013;3

    Google Scholar 

  7. Ramirez-Castaneda J, Jankovic J. Long-term efficacy, safety, and side effect profile of botulinum toxin in dystonia: a 20-year follow-up. Toxicon. 2014;90:344–8.

    Article  CAS  PubMed  Google Scholar 

  8. Fahn S, Bressman SB, Marsden CD. Classification of dystonia. Adv Neurol. 1998;78:1–10.

    Article  CAS  PubMed  Google Scholar 

  9. Stamelou M, Edwards MJ, Hallett M, Bhatia KP. The non-motor syndrome of primary dystonia: clinical and pathophysiological implications. Brain. 2012;135:1668–81.

    Article  PubMed  Google Scholar 

  10. Defazio G, Abbruzzese G, Livrea P, Berardelli A. Epidemiology of primary dystonia. Lancet Neurol. 2004;3:673–8.

    Article  PubMed  Google Scholar 

  11. Jankovic J, Leder S, Warner D, Schwartz K. Cervical dystonia: clinical findings and associated movement disorders. Neurology. 1991;41:1088–91.

    Article  CAS  PubMed  Google Scholar 

  12. Steeves TD, Day L, Dykeman J, Jette N, Pringsheim T. The prevalence of primary dystonia: a systematic review and meta-analysis. Mov Disord. 2012;27:1789–96.

    Article  PubMed  Google Scholar 

  13. Abbruzzese G, Berardelli A, Girlanda P, et al. Long-term assessment of the risk of spread in primary late-onset focal dystonia. J Neurol Neurosurg Psychiatry. 2008;79:392–6.

    Article  CAS  PubMed  Google Scholar 

  14. Valls-Sole J, Defazio G. Blepharospasm: update on epidemiology, clinical aspects, and pathophysiology. Front Neurol. 2016;7:45.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Pandey S. A practical approach to management of focal hand dystonia. Ann Indian Acad Neurol. 2015;18:146–53.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Jedynak PC, Tranchant C, de Beyl DZ. Prospective clinical study of writer's cramp. Mov Disord. 2001;16:494–9.

    Article  CAS  PubMed  Google Scholar 

  17. Eskow Jaunarajs KL, Bonsi P, Chesselet MF, Standaert DG, Pisani A. Striatal cholinergic dysfunction as a unifying theme in the pathophysiology of dystonia. Prog Neurobiol. 2015;127–128:91–107.

    Article  PubMed  Google Scholar 

  18. LeDoux MS, Brady KA. Secondary cervical dystonia associated with structural lesions of the central nervous system. Mov Disord. 2003;18:60–9.

    Article  PubMed  Google Scholar 

  19. Peller M, Zeuner KE, Munchau A, et al. The basal ganglia are hyperactive during the discrimination of tactile stimuli in writer’s cramp. Brain. 2006;129:2697–708.

    Article  CAS  PubMed  Google Scholar 

  20. Lera G, Bhatia K, Marsden CD. Dystonia as the major manifestation of Leigh’s syndrome. Mov Disord. 1994;9:642–9.

    Article  CAS  PubMed  Google Scholar 

  21. Bradley D, Whelan R, Walsh R, et al. Temporal discrimination threshold: VBM evidence for an endophenotype in adult onset primary torsion dystonia. Brain. 2009;132:2327–35.

    Article  CAS  PubMed  Google Scholar 

  22. Argyelan M, Carbon M, Niethammer M, et al. Cerebellothalamocortical connectivity regulates penetrance in dystonia. J Neurosci. 2009;29:9740–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Naudon L, Delfs JM, Clavel N, Lorden JF, Chesselet MF. Differential expression of glutamate decarboxylase messenger RNA in cerebellar Purkinje cells and deep cerebellar nuclei of the genetically dystonic rat. Neuroscience. 1998;82:1087–94.

    Article  CAS  PubMed  Google Scholar 

  24. Raike RS, Jinnah HA, Hess EJ. Animal models of generalized dystonia. NeuroRx. 2005;2:504–12.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Albright AL, Ferson SS. Intraventricular baclofen for dystonia: techniques and outcomes. Clinical article. J Neurosurg Pediatr. 2009;3:11–4.

    Article  PubMed  Google Scholar 

  26. 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.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. 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;86:1818–26.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  28. Hallett M. Neurophysiology of dystonia: the role of inhibition. Neurobiol Dis. 2011;42:177–84.

    Article  PubMed  Google Scholar 

  29. Morgante F, Klein C. Dystonia. Continuum (Minneap Minn). 2013;19:1225–41.

    Google Scholar 

  30. Quartarone A, Pisani A. Abnormal plasticity in dystonia: disruption of synaptic homeostasis. Neurobiol Dis. 2011;42:162–70.

    Article  PubMed  Google Scholar 

  31. Quartarone A, Hallett M. Emerging concepts in the physiological basis of dystonia. Mov Disord. 2013;28:958–67.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Burke RE, Fahn S, Marsden CD. Torsion dystonia: a double-blind, prospective trial of high-dosage trihexyphenidyl. Neurology. 1986;36:160–4.

    Article  CAS  PubMed  Google Scholar 

  33. Greene P, Shale H, Fahn S. Analysis of open-label trials in torsion dystonia using high dosages of anticholinergics and other drugs. Mov Disord. 1988;3:46–60.

    Article  CAS  PubMed  Google Scholar 

  34. Bressman SB, Greene PE. Treatment of hyperkinetic movement disorders. Neurol Clin. 1990;8:51–75.

    CAS  PubMed  Google Scholar 

  35. Cao S, Hewett JW, Yokoi F, et al. Chemical enhancement of torsinA function in cell and animal models of torsion dystonia. Dis Model Mech. 2010;3:386–96.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. Loscher W, Richter A. Piracetam and levetiracetam, two pyrrolidone derivatives, exert antidystonic activity in a hamster model of paroxysmal dystonia. Eur J Pharmacol. 2000;391:251–4.

    Article  CAS  PubMed  Google Scholar 

  37. Aoki KR, Guyer B. Botulinum toxin type A and other botulinum toxin serotypes: a comparative review of biochemical and pharmacological actions. Eur J Neurol. 2001;8(Suppl 5):21–9.

    Article  PubMed  Google Scholar 

  38. Truong D, Dressler D, Hallett M. Manual of botulinum toxin therapy. Cambridge, UK. New York: Cambridge University Press; 2009.

    Google Scholar 

  39. Shanker V, Bressman SB. Diagnosis and Management of Dystonia. Continuum (Minneap Minn). 2016;22:1227–45.

    Google Scholar 

  40. Walker TJ, Dayan SH. Comparison and overview of currently available neurotoxins. J Clin Aesthet Dermatol. 2014;7:31–9.

    PubMed  PubMed Central  Google Scholar 

  41. Fraint A, Vittal P, Comella C. Considerations on patient-related outcomes with the use of botulinum toxins: is switching products safe? Ther Clin Risk Manag. 2016;12:147–54.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Kollewe K, Mohammadi B, Kohler S, Pickenbrock H, Dengler R, Dressler D. Blepharospasm: long-term treatment with either Botox(R), Xeomin(R) or Dysport(R). J Neural Transm (Vienna). 2015;122:427–31.

    Article  CAS  Google Scholar 

  43. Comella CL, Jankovic J, Truong DD, Hanschmann A, Grafe S, Group USXCDS. Efficacy and safety of incobotulinumtoxinA (NT 201, XEOMIN(R), botulinum neurotoxin type A, without accessory proteins) in patients with cervical dystonia. J Neurol Sci. 2011;308:103–9.

    Article  CAS  PubMed  Google Scholar 

  44. Evidente VG, Fernandez HH, LeDoux MS, et al. A randomized, double-blind study of repeated incobotulinumtoxinA (Xeomin((R))) in cervical dystonia. J Neural Transm (Vienna). 2013;120:1699–707.

    Article  CAS  Google Scholar 

  45. Charles D, Brashear A, Hauser RA, et al. Efficacy, tolerability, and immunogenicity of onabotulinumtoxina in a randomized, double-blind, placebo-controlled trial for cervical dystonia. Clin Neuropharmacol. 2012;35:208–14.

    Article  CAS  PubMed  Google Scholar 

  46. Greene P, Kang U, Fahn S, Brin M, Moskowitz C, Flaster E. Double-blind, placebo-controlled trial of botulinum toxin injections for the treatment of spasmodic torticollis. Neurology. 1990;40:1213–8.

    Article  CAS  PubMed  Google Scholar 

  47. Hallett M, Albanese A, Dressler D, et al. Evidence-based review and assessment of botulinum neurotoxin for the treatment of movement disorders. Toxicon. 2013;67:94–114.

    Article  CAS  PubMed  Google Scholar 

  48. Kruisdijk JJ, Koelman JH, Ongerboer de Visser BW, de Haan RJ, Speelman JD. Botulinum toxin for writer's cramp: a randomised, placebo-controlled trial and 1-year follow-up. J Neurol Neurosurg Psychiatry. 2007;78:264–70.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Chapman MA, Barron R, Tanis DC, Gill CE, Charles PD. Comparison of botulinum neurotoxin preparations for the treatment of cervical dystonia. Clin Ther. 2007;29:1325–37.

    Article  CAS  PubMed  Google Scholar 

  50. Grigoriu AI, Dinomais M, Remy-Neris O, Brochard S. Impact of injection-guiding techniques on the effectiveness of botulinum toxin for the treatment of focal spasticity and dystonia: a systematic review. Arch Phys Med Rehabil. 2015;96:2067–78. e2061

    Article  PubMed  Google Scholar 

  51. Delnooz CC, Veugen LC, Pasman JW, Lapatki BG, van Dijk JP, van de Warrenburg BP. The clinical utility of botulinum toxin injections targeted at the motor endplate zone in cervical dystonia. Eur J Neurol. 2014;21:1486–e98.

    Article  CAS  PubMed  Google Scholar 

  52. Jankovic J, Adler CH, Charles D, et al. Primary results from the cervical dystonia patient registry for observation of onabotulinumtoxina efficacy (CD PROBE). J Neurol Sci. 2015;349:84–93.

    Article  PubMed  Google Scholar 

  53. Lungu C, Considine E, Zahir S, Ponsati B, Arrastia S, Hallett M. Pilot study of topical acetyl hexapeptide-8 in the treatment for blepharospasm in patients receiving botulinum toxin therapy. Eur J Neurol. 2013;20:515–8.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Rush University Medical Center – Section of Movement Disorders, 1725 W Harrison Street, Suite 755, Chicago, IL, 60612, USA

    Avram Fraint M.D.

  2. Rush University Medical Center – Section of Movement Disorders, 1725 W Harrison, Suite 755, Chicago, IL, 60612, USA

    Cynthia Comella M.D.

Authors
  1. Avram Fraint M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Cynthia Comella M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Avram Fraint M.D. .

Editor information

Editors and Affiliations

  1. Emeritus Professor of Neurology, Yale University, New Haven, Connecticut, USA

    Bahman Jabbari

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG

About this chapter

Cite this chapter

Fraint, A., Comella, C. (2018). Botulinum Toxin Treatment of Primary Dystonia. In: Jabbari, B. (eds) Botulinum Toxin Treatment in Clinical Medicine. Springer, Cham. https://doi.org/10.1007/978-3-319-56038-0_2

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-56038-0_2

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-56037-3

  • Online ISBN: 978-3-319-56038-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation