Journal of Neurology

, Volume 263, Issue 11, pp 2319–2326

Long-term results of deep brain stimulation in a cohort of eight children with isolated dystonia

  • P. Krause
  • K. Lauritsch
  • A. Lipp
  • A. Horn
  • B. Weschke
  • A. Kupsch
  • K. L. Kiening
  • G.-H. Schneider
  • A. A. Kühn
Original Communication

DOI: 10.1007/s00415-016-8253-6

Cite this article as:
Krause, P., Lauritsch, K., Lipp, A. et al. J Neurol (2016) 263: 2319. doi:10.1007/s00415-016-8253-6

Abstract

Pallidal deep brain stimulation (DBS) is an established treatment for patients with severe isolated dystonia. However, clinical evidence for the long-term use of DBS in children is limited and controlled trials have not yet been conducted. Here, we provide the long-term results of up to 13 years of pallidal DBS in eight pediatric patients with generalized idiopathic or hereditary isolated dystonia (five males, mean age at surgery 12.5 ± 3.5 years), as assessed by retrospective video rating. Video rating was performed at three time points: pre-operative, 1-year short-term follow-up (1y-FU) and long-term last FU (LT-FU, up to 13 years). Symptom severity and disability were assessed using the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS). Disability scores were obtained from clinical charts and during the last FU. The mean improvement in BFMDRS motor score was 54.4 ± 8.9 % at 1y-FU and 42.9 ± 11.6 % at LT-FU; the disability scores improved by 59.8 ± 10.3 and 63.3 ± 7.8 %, respectively. Electrode dislocation was noted in one patient and implantable pulse generator dislocation in another, both requiring surgical intervention; no further serious adverse events occurred. Our study presents the first blinded video rating assessment of the short- and long-term effects of pallidal DBS in children with idiopathic or hereditary isolated dystonia. Results confirm that pallidal DBS is a safe and efficacious long-term treatment in children, with overall motor improvement similar to that described in controlled trials in adults.

Keywords

DYT1 dystonia Pallidal DBS Long-term effects Idiopathic dystonia 

Supplementary material

View video

Video legend: Segment 1: Patient 2 with generalized dystonia with predominant phasic movements of the head as well as right upper extremity and dystonic gait disorder with bending of the trunk, inward rotation and involuntary flexion of the legs while walking. At no time did she experience speech and/or swallowing problems. Segment 2: After 21 months of pallidal DBS, phasic involuntary movements of the head and right arm were almost completely suppressed, along with significant improvement of cervical and truncal dystonia as well as relevant reduction of dystonia of the upper extremities and dystonic gait. Segment 3: DBS effects remaining stable for more than 13 years, with sustained relief of disability and cessation of the phasic involuntary head and upper limb movements. Mild residual dystonia of the limbs results in minor limitations of fine motor skills and gait. (WMV 43560 kb)

Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  1. 1.Department of NeurologyCharité, University Medicine BerlinBerlinGermany
  2. 2.Department of NeuropediatricsCharité, University Medicine BerlinBerlinGermany
  3. 3.Department of NeurosurgeryCharité, University Medicine BerlinBerlinGermany
  4. 4.Department of Neurology and Stereotactic NeurosurgeryUniversity Medicine of MagdeburgMagdeburgGermany
  5. 5.Department of Neurosurgery, Division of Stereotactic NeurosurgeryUniversity Medicine HeidelbergHeidelbergGermany