Deep Brain Stimulation for Pediatric Dystonia

  • Michelle A. WedemeyerEmail author
  • Mark A. Liker
Living reference work entry


Dystonia is defined as a movement disorder in which “involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both.” Dystonia may be classified as primary or secondary. Primary dystonia is characterized by isolated dystonia and is the result of number of hereditary mutations. Secondary or symptomatic dystonia is acquired from any number of insults to the brain, most commonly cerebral palsy, kernicterus, or other toxic/metabolic insults to the brain. Secondary dystonias present earlier in life and are associated with other neurologic symptoms including epilepsy, developmental delay, spasticity, and hypertonia.

Any child presenting with a clinical exam concerning for dystonia should be referred to a neurologist specializing in movement disorders as dystonia is associated with the development of skeletal abnormalities in the growing child. Initial management is medical; however, some patients are refractory to medical management and may be candidates for surgical evaluation.

Deep brain stimulation (DBS) is the treatment of choice for dystonia and involves stereotactic placement of neurostimulators powered by an implantable pulse generator into the basal ganglia. Although patients with primary dystonia may have marked improvements with stimulation, patients with secondary or progressive dystonias characteristically have more variable responses and on average show more modest improvements on clinical measures of dystonia. Despite its invasive nature, this procedure is relatively safe with low mortality although reduction in the rate of surgical site infections, the most common complication, remains an active area of research.


Dystonia Deep brain stimulation Pallidotomy Thalamotomy Basal ganglia 


  1. Air EL, Ostrem JL, Sanger TD, Starr PA (2011) Deep brain stimulation in children: experience and technical pearls. J Neurosurg Pediatr 8:566–574CrossRefPubMedGoogle Scholar
  2. Albanese A, Bhatia K, Bressman SB, Delong MR, Fahn S, Fung VS et al (2013) Phenomenology and classification of dystonia: a consensus update. Mov Disord 28:863–873CrossRefPubMedPubMedCentralGoogle Scholar
  3. Benabid AL, Pollak P, Gervason C, Hoffmann D, Gao DM, Hommel M et al (1991) Long-term suppression of tremor by chronic stimulation of the ventral intermediate thalamic nucleus. Lancet 337:403–406CrossRefPubMedGoogle Scholar
  4. Benabid AL, Pollak P, Gao D, Hoffmann D, Limousin P, Gay E et al (1996) Chronic electrical stimulation of the ventralis intermedius nucleus of the thalamus as a treatment of movement disorders. J Neurosurg 84:203–214CrossRefPubMedGoogle Scholar
  5. Dijk JM, Tijssen MA (2010) Management of patients with myoclonus: available therapies and the need for an evidence-based approach. Lancet Neurol 9:1028–1036CrossRefPubMedGoogle Scholar
  6. Eltahawy HA, Saint-Cyr J, Giladi N, Lang AE, Lozano AM (2004) Primary dystonia is more responsive than secondary dystonia to pallidal interventions: outcome after pallidotomy or pallidal deep brain stimulation. Neurosurgery 54:613–619.; discussion 619-621CrossRefPubMedGoogle Scholar
  7. Elze MC, Gimeno H, Tustin K, Baker L, Lumsden DE, Hutton JL et al (2016) Burke-Fahn-Marsden dystonia severity, gross motor, manual ability, and communication function classification scales in childhood hyperkinetic movement disorders including cerebral palsy: a ‘Rosetta Stone’ study. Dev Med Child Neurol 58:145–153CrossRefPubMedGoogle Scholar
  8. Gimeno H, Lin JP (2017) The international classification of functioning (ICF) to evaluate deep brain stimulation neuromodulation in childhood dystonia-hyperkinesia informs future clinical & research priorities in a multidisciplinary model of care. Eur J Paediatr Neurol 21:147–167CrossRefPubMedGoogle Scholar
  9. Gimeno H, Tustin K, Selway R, Lin JP (2012) Beyond the Burke-Fahn-Marsden dystonia rating scale: deep brain stimulation in childhood secondary dystonia. Eur J Paediatr Neurol 16:501–508CrossRefPubMedGoogle Scholar
  10. Gimeno H, Gordon A, Tustin K, Lin JP (2013) Functional priorities in daily life for children and young people with dystonic movement disorders and their families. Eur J Paediatr Neurol 17:161–168CrossRefPubMedGoogle Scholar
  11. Gimeno H, Tustin K, Lumsden D, Ashkan K, Selway R, Lin JP (2014) Evaluation of functional goal outcomes using the Canadian occupational performance measure (COPM) following deep brain stimulation (DBS) in childhood dystonia. Eur J Paediatr Neurol 18:308–316CrossRefPubMedGoogle Scholar
  12. Hudson VE, Elniel A, Ughratdar I, Zebian B, Selway R, Lin JP (2017) A comparative historical and demographic study of the neuromodulation management techniques of deep brain stimulation for dystonia and cochlear implantation for sensorineural deafness in children. Eur J Paediatr Neurol 21:122–135CrossRefPubMedGoogle Scholar
  13. Kaminska M, Perides S, Lumsden DE, Nakou V, Selway R, Ashkan K et al (2017) Complications of deep brain stimulation (DBS) for dystonia in children - the challenges and 10 year experience in a large paediatric cohort. Eur J Paediatr Neurol 21:168–175CrossRefPubMedGoogle Scholar
  14. Karas PJ, Mikell CB, Christian E, Liker MA, Sheth SA (2013) Deep brain stimulation: a mechanistic and clinical update. Neurosurg Focus 35:E1CrossRefPubMedGoogle Scholar
  15. 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 151:473–478.; discussion 478CrossRefPubMedGoogle Scholar
  16. Keen JR, Przekop A, Olaya JE, Zouros A, Hsu FP (2014) Deep brain stimulation for the treatment of childhood dystonic cerebral palsy. J Neurosurg Pediatr 14:585–593CrossRefPubMedGoogle Scholar
  17. Koy A, Hellmich M, Pauls KA, Marks W, Lin JP, Fricke O et al (2013) Effects of deep brain stimulation in dyskinetic cerebral palsy: a meta-analysis. Mov Disord 28:647–654CrossRefPubMedGoogle Scholar
  18. Koy A, Lin JP, Sanger TD, Marks WA, Mink JW, Timmermann L (2016) Advances in management of movement disorders in children. Lancet Neurol 15:719–735CrossRefPubMedGoogle Scholar
  19. Kupsch A, Benecke R, Muller J, Trottenberg T, Schneider GH, Poewe W et al (2006) Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med 355:1978–1990CrossRefPubMedGoogle Scholar
  20. Loher TJ, Pohle T, Krauss JK (2004) Functional stereotactic surgery for treatment of cervical dystonia: review of the experience from the lesional era. Stereotact Funct Neurosurg 82:1–13CrossRefPubMedGoogle Scholar
  21. Lumsden DE, Kaminska M, Gimeno H, Tustin K, Baker L, Perides S et al (2013) Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia. Dev Med Child Neurol 55:567–574CrossRefPubMedGoogle Scholar
  22. Lumsden DE, Gimeno H, Tustin K, Kaminska M, Lin JP (2015) Interventional studies in childhood dystonia do not address the concerns of children and their carers. Eur J Paediatr Neurol 19:327–336CrossRefPubMedGoogle Scholar
  23. Lumsden DE, Gimeno H, Elze M, Tustin K, Kaminska M, Lin JP (2016) Progression to musculoskeletal deformity in childhood dystonia. Eur J Paediatr Neurol 20:339–345CrossRefPubMedGoogle Scholar
  24. Marks WA, Honeycutt J, Acosta F, Reed M (2009) Deep brain stimulation for pediatric movement disorders. Semin Pediatr Neurol 16:90–98CrossRefPubMedGoogle Scholar
  25. Marks W, Bailey L, Reed M, Pomykal A, Mercer M, Macomber D et al (2013) Pallidal stimulation in children: comparison between cerebral palsy and DYT1 dystonia. J Child Neurol 28:840–848CrossRefPubMedGoogle Scholar
  26. Markun LC, Starr PA, Air EL, Marks WJ Jr, Volz MM, Ostrem JL (2012) Shorter disease duration correlates with improved long-term deep brain stimulation outcomes in young-onset DYT1 dystonia. Neurosurgery 71:325–330CrossRefPubMedGoogle Scholar
  27. McClelland VM, Valentin A, Rey HG, Lumsden DE, Elze MC, Selway R et al (2016) Differences in globus pallidus neuronal firing rates and patterns relate to different disease biology in children with dystonia. J Neurol Neurosurg Psychiatry 87:958–967CrossRefPubMedPubMedCentralGoogle Scholar
  28. Odding E, Roebroeck ME, Stam HJ (2006) The epidemiology of cerebral palsy: incidence, impairments and risk factors. Disabil Rehabil 28:183–191CrossRefPubMedGoogle Scholar
  29. Olaya JE, Christian E, Ferman D, Luc Q, Krieger MD, Sanger TD et al (2013) Deep brain stimulation in children and young adults with secondary dystonia: the Children’s Hospital Los Angeles experience. Neurosurg Focus 35:E7CrossRefPubMedGoogle Scholar
  30. Parr JR, Green AL, Joint C, Andrew M, Gregory RP, Scott RB et al (2007) Deep brain stimulation in childhood: an effective treatment for early onset idiopathic generalised dystonia. Arch Dis Child 92:708–711CrossRefPubMedPubMedCentralGoogle Scholar
  31. Romito LM, Zorzi G, Marras CE, Franzini A, Nardocci N, Albanese A (2015) Pallidal stimulation for acquired dystonia due to cerebral palsy: beyond 5 years. Eur J Neurol 22:426–e432CrossRefPubMedGoogle Scholar
  32. Sanger TD, Delgado MR, Gaebler-Spira D, Hallett M, Mink JW (2003) Task force on childhood motor D: classification and definition of disorders causing hypertonia in childhood. Pediatrics 111:e89–e97CrossRefPubMedGoogle Scholar
  33. Vidailhet M, Vercueil L, Houeto JL, Krystkowiak P, Benabid AL, Cornu P et al (2005) Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med 352:459–467CrossRefPubMedGoogle Scholar
  34. Vidailhet M, Yelnik J, Lagrange C, Fraix V, Grabli D, Thobois S et al (2009) Bilateral pallidal deep brain stimulation for the treatment of patients with dystonia-choreoathetosis cerebral palsy: a prospective pilot study. Lancet Neurol 8:709–717CrossRefPubMedGoogle Scholar
  35. Volkmann J, Wolters A, Kupsch A, Muller J, Kuhn AA, Schneider GH et al (2012) Pallidal deep brain stimulation in patients with primary generalised or segmental dystonia: 5-year follow-up of a randomised trial. Lancet Neurol 11:1029–1038CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of NeurosurgeryUniversity of Southern California, Keck School of MedicineLos AngelesUSA
  2. 2.Division of NeurosurgeryChildren’s Hospital of Los AngelesLos AngelesUSA

Section editors and affiliations

  • Freeman Miller
    • 1
  1. 1.AI DuPont Hospital for ChildrenWilmingtonUSA

Personalised recommendations