Functional Neurosurgery for Secondary Dystonia: Indications and Long-Term Results

  • Jairo Alberto Espinoza MartinezEmail author
  • Oscar Andres Escobar Vidarte
  • Gabriel Arango Uribe
Conference paper
Part of the Acta Neurochirurgica Supplement book series (NEUROCHIRURGICA, volume 117)


Dystonia is a movement disorder characterized by patterned, repetitive, phasic, or tonic sustained muscle contractions that produce abnormal, often twisting, postures or repetitive movements. When the disorder is genetic or the cause is unknown and dystonia is the sole feature, the disease is called primary or idiopathic, conversely secondary dystonia (SD) may be caused by various brain insults. Both primary dystonia and SD have been notorious for their poor response to medical treatment. Today, stereotactic neurosurgical procedures are offered to improve the disability and quality of life of patients who do not respond to medical therapy. However, SD shows less and more variable results than primary dystonia to neurosurgical procedures, the benefits of ablative or deep brain stimulation (DBS) procedures in basal structures being still subject to debate and much harder to fully appreciate. In this work, the authors show a 33-patient series with secondary dystonia, separating the statistic and clinical analysis into several etiology groups: perinatal insults, tardive syndromes, genetic syndromes, and posttraumatic. In these groups, we show the mean BFM score improvement in the different patient series, comparing our results with world literature, and finally propose a classification system for bettering the clinical approach in surgery decision when this is indicated.


Secondary dystonia Deep brain stimula-tion Intrathecal baclofen Brain motor circuits 


Conflict of Interest

We declare that we have no conflict of interest.


  1. 1.
    Capelle H, Krauss J (2009) Functional stereotactic procedures for treatment of secondary dystonia. In: Lozano A, Gildenberg P, Tasker R (eds) Textbook of stereotactic and functional neurosurgery. Springer, Berlin, pp 1835–1856CrossRefGoogle Scholar
  2. 2.
    Eltahawy H, Saint-Cyr J, Giladi N, Lang A, Lozano A (2004) Primary dystonia is more responsive than secondary dystonia to pallidal interventions: outcome after pallidotomy or pallidal deep brain stimulation. Neurosurgery 54:613–621PubMedCrossRefGoogle Scholar
  3. 3.
    Geyer HL, Bressman SB (2007) Diagnosis of dystonia. In: Warner TT, Bressman SB (eds) Clinical diagnosis and management of dystonia. Informa healthcare, London, pp 1–14CrossRefGoogle Scholar
  4. 4.
    Iacono RP, Kuniyoshi SM, Lonser RR, Maeda G, Inae AM, Ashwal S (1996) Simultaneous bilateral pallidoansotomy for idiopathic dystonia musculorum deformans. Pediatr Neurol 14:145–148PubMedCrossRefGoogle Scholar
  5. 5.
    Krause M, Fogel W, Kloss M, Rasche D, Volkmann J (2004) Pallidal stimulation for dystonia. Neurosurgery 55:1361–1370PubMedCrossRefGoogle Scholar
  6. 6.
    Iacono RP, Kuniyoshi SM, Schoonenberg T (1998) Experience with stereotactics for dystonia: case examples. Adv Neurol 78:221–226PubMedGoogle Scholar
  7. 7.
    Lin JJ, Lin SZ, Lin GY, Chang DC, Lee CC (1998) Application of bilateral sequential pallidotomy to treat a patient with generalized dystonia. Eur Neurol 40:108–110PubMedGoogle Scholar
  8. 8.
    Lin JJ, Lin GY, Shih C, Lin SZ, Chang DC, Lee CC (1999) Benefit of bilateral pallidotomy in the treatment of generalized dystonia: case report. J Neurosurg 90:974–976PubMedCrossRefGoogle Scholar
  9. 9.
    Lozano AM, Kumar R, Gross RE, Giladi N, Hutchison WD, Dostrovsky JO, Lang AE (1997) Globus pallidus internus pallidotomy for generalized dystonia. Mov Disord 12:865–870PubMedCrossRefGoogle Scholar
  10. 10.
    Vitek JL (1998) Surgery for dystonia. Neurosurg Clin N Am 9:345–366PubMedGoogle Scholar
  11. 11.
    Vitek JL, Zhang J, Evatt M, Mewes K, DeLong MR, Hashimoto T, Triche S, Bakay RAE (1998) GPi pallidotomy for dystonia: clinical outcome and neuronal activity. Adv Neurol 78:211–219PubMedGoogle Scholar
  12. 12.
    Krauss JK, Yianni J, Loher TJ, Aziz TZ (2004) Deep brain stimulation for dystonia. J Clin Neurophysiol 21:18–30PubMedCrossRefGoogle Scholar
  13. 13.
    Welter M, Grabli D, Vidailhet M (2010) Deep brain stimulation for hyperkinetic disorders: dystonia, tardive dyskinesia and tics. Curr Opin Neurol 23:420–425PubMedGoogle Scholar
  14. 14.
    Vercueil L, Pollak P, Fraix V, Caputo E, Moro E, Benazzouz A et al (2001) Deep brain stimulation in the treatment of severe dystonia. J Neurol 248:695–700PubMedCrossRefGoogle Scholar
  15. 15.
    Cif L, El Fertit H, Vayssiere N, Hemm S, Hardouin E, Gannau A, Tuffery S, Coubes P (2003) Treatment of dystonic syndromes by chronic electrical stimulation of the internal globus pallidus. J Neurosurg Sci 47:52–55PubMedGoogle Scholar
  16. 16.
    Aziz T, Green A (2009) Dystonia: a surgeon’s perspective. Parkinsonism Relat Disord 15S(3):S75–S80CrossRefGoogle Scholar
  17. 17.
    Katsakiori PF, Kefalopoulou Z, Markaki E, Paschali A, Ellul J, Kagadis GC et al (2009) Deep brain stimulation for secondary dystonia: results in 8 patients. Acta Neurochir 151(5):473–478PubMedCrossRefGoogle Scholar
  18. 18.
    Lin JJ, Lin SZ, Chang DC (1999) Pallidotomy and generalized dystonia. Mov Disord 14:1057–1059PubMedCrossRefGoogle Scholar
  19. 19.
    Ondo WG, Krauss JK (2003) Surgical therapies for dystonia. In: Brin MF, Comella C, Jankovic J (eds) Dystonia monograph. Lippincott, Williams & Wilkins, Philadelphia, pp 125–147Google Scholar
  20. 20.
    Trottenberg T, Volkmann J, Deuschl G, Kuhn AA, Schneider GH, Muller J et al (2005) Treatment of severe tardive dystonia with pallidal deep brain stimulation. Neurology 64:344–346PubMedCrossRefGoogle Scholar
  21. 21.
    Arnone D, Hansen L, Kerr JS (2002) Acute dystonic reaction in an elderly patient with mood disorder after titration of paroxetine: possible mechanisms and implications for clinical care. J Psychopharmacol 16:395–397PubMedCrossRefGoogle Scholar
  22. 22.
    Sako W, Goto S, Shimazu H, Murase N, Matsuzaki K, Tamura T et al (2008) Bilateral deep brain stimulation of the globus pallidus internus in tardive dystonia. Mov Disord 23:1929–1931PubMedCrossRefGoogle Scholar
  23. 23.
    Damier P, Thobois S, Witjas T, Cuny E, Derost P, Raoul S et al (2007) French Stimulation for Tardive Dyskinesia (STARDYS) Study Group. Bilateral deep brain stimulation of the globus pallidus to treat tardive dyskinesia. Arch Gen Psychiatry 64:170–176PubMedCrossRefGoogle Scholar
  24. 24.
    Vidailhet M, Lagrannge C, Welter ML, Krack P, Krystowiak P, Burbaud P et al (2008) Bilateral pallidal stimulation in generalized dystonia related to post-anoxic birth injury: multicentre study. Mov Disord Suppl 1:162Google Scholar
  25. 25.
    Vidailhet M, Yelnik J, Lagrange C et al (2009) Bilateral pallidal deep brain stimulation for the treatment of patients with dystonia-choreoathetosis cerebral palsy: a prospective pilot study. Lancet Neurol 8:709–717PubMedCrossRefGoogle Scholar
  26. 26.
    Krauss JK, Loher TJ, Weigel R, Capelle HH, Weber S, Burgunder JM (2003) Chronic stimulation of the globus pallidus internus for treatment of non-DYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg 98:785–792PubMedCrossRefGoogle Scholar
  27. 27.
    Loher TJ, Hasdemir MG, Burgunder JM, Krauss JK (2000) Long-term follow-up study of chronic globus pallidus internus stimulation for posttraumatic hemidystonia. J Neurosurg 92:457–460PubMedCrossRefGoogle Scholar
  28. 28.
    Yianni J, Bain P, Giladi N, Auca M, Gregory R, Joint C, Nandi D, Stein J, Scott R, Aziz T (2003) Globus pallidus internus deep brain stimulation for dystonic conditions: a prospective audit. Mov Disord 18:436–442PubMedCrossRefGoogle Scholar
  29. 29.
    Motta F, Antonello C, Stignani C (2009) Upper limbs function after intrathecal baclofen therapy in children with secondary dystonia. J Pediatr Orthop 29:817–821PubMedCrossRefGoogle Scholar
  30. 30.
    Butler C, Campbell S (2000) Evidence of the effects of intrathecal baclofen for spastic and dystonic cerebral palsy. Dev Med Child Neurol 42(9):634–645PubMedCrossRefGoogle Scholar
  31. 31.
    Albright AL, Ferson SS (2006) Intrathecal baclofen therapy in children. Neurosurg Focus 21(2):e3PubMedCrossRefGoogle Scholar
  32. 32.
    Narayan RK, Loubser PG, Jankovic J et al (1991) Intrathecal baclofen for intractable axial dystonia. Neurology 41(7):1141–1142PubMedCrossRefGoogle Scholar
  33. 33.
    Penn RD, Gianino JM, York MM (1995) Intrathecal baclofen for motor disorders. Mov Disord 10(5):675–677PubMedCrossRefGoogle Scholar
  34. 34.
    Ford B, Green P, Louis E, Petzinger G, Bressman S, Goodman R et al (1996) Use of intrathecal baclofen in the treatment of patients with dystonia. Arch Neurol 53:1241–1246PubMedCrossRefGoogle Scholar
  35. 35.
    Albright AL, Barry MJ, Shafron DH, Ferson SS (2001) Intrathecal baclofen for generalized dystonia. Dev Med Child Neurol 43:652–657PubMedCrossRefGoogle Scholar
  36. 36.
    Albright AL (1996) Intrathecal baclofen in cerebral palsy movement disorders. J Child Neurol 11:S29–S35PubMedCrossRefGoogle Scholar
  37. 37.
    Albright AL, Barry MJ, Painter MJ, Shultz B (1998) Infusion of intrathecal baclofen for generalized dystonia in cerebral palsy. J Neurosurg 88:73–76PubMedCrossRefGoogle Scholar
  38. 38.
    Motta F, Stignani C, Antonello CE (2008) Effect of intrathecal baclofen on dystonia in children with cerebral palsy and the use of functional scales. J Pediatr Orthop 28:213–217PubMedCrossRefGoogle Scholar
  39. 39.
    Ward A, Hayden S, Dexter M, Scheinberg A (2009) Continuous intrathecal baclofen for children with spasticity and/or dystonia: goal attainment and complications associated with treatment. J Paediatr Child Health 45:720–726PubMedCrossRefGoogle Scholar
  40. 40.
    Albright AL, Barry MJ, Fasick P, Barron W, Shultz B (1996) Continuous intrathecal baclofen infusion for symptomatic generalized dystonia. Neurosurgery 38:934–939PubMedCrossRefGoogle Scholar
  41. 41.
    Albright AL (2007) Intrathecal baclofen for childhood hypertonia. Childs Nerv Syst 23:971–979PubMedCrossRefGoogle Scholar
  42. 42.
    Albright AL (1996) Baclofen in the treatment of cerebral palsy. J Child Neurol 11(2):77–83PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Wien 2013

Authors and Affiliations

  • Jairo Alberto Espinoza Martinez
    • 1
    • 2
    • 3
    Email author
  • Oscar Andres Escobar Vidarte
    • 1
    • 2
    • 3
  • Gabriel Arango Uribe
    • 1
    • 2
    • 3
  1. 1.Movement Disorders and Pain Management Center, CIMADBogotaColombia
  2. 2.Marly ClinicBogotaColombia
  3. 3.San Jose Children’s HospitalBogotaColombia

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