Session Information
Date: Thursday, June 23, 2016
Session Title: Pediatric movement disorder
Session Time: 12:00pm-1:30pm
Location: Exhibit Hall located in Hall B, Level 2
Objective: We present long-term results up to 13 years in a consecutive series of 8 pediatric patients assessed with blinded video rating.
Background: : The efficacy of pallidal deep brain stimulation (DBS) in patients with severe idiopathic or inherited isolated dystonia has been proven in sham controlled trials for adults. Although clinical observations in children with DBS report even higher improvement rates, clinical evidence for the long-term use of DBS in children is limited and controlled trials have not been conducted so far.
Methods: Retrospective video rating of 8 pediatric patients with generalized isolated dystonia (5 male, mean age at surgery 12.5±3.5 years) who underwent bilateral DBS in the globus pallidus internus. Video rating was performed at three time points: pre-operative, short-term follow-up (FU) and last FU (up to 13 years). Symptom severity was assessed using the Burke-Fahn-Marsden-Dystonia-Rating-Scale (BFMDRS). Disability scores were obtained from clinical charts and during last FU.
Results: The mean improvement in BFMDRS-motor score was 54.4±8.9% at short-term FU and 42.9±11.6% at last FU; and the disability scores improved by 59.8±10.3% and 63.3±7.8% respectively. Subscores show consistent long-term improvement for limb dystonia and reduced efficacy for laryngeal and cervical dystonia in our patients, similar to previous reports in adults. Electrode dislocation was noted in one and impulse generator (IPG) dislocation in another patient which both needed surgical intervention; no further serious adverse events occurred in the remaining patients.
Conclusions: Our study extends the rare previous reports on DBS in children, presents for the first time a blinded video assessment of short- and long-term effects of pallidal DBS in children with isolated dystonia and confirms pallidal DBS as a safe and efficacious long-term treatment for these patients.
To cite this abstract in AMA style:
P. Krause, K. Lauritsch, A. Lipp, A. Horn, B. Weschke, A. Kupsch, K. Kiening, G.H. Schneider, A. Kühn. Long-term results of pallidal deep brain stimulation in a cohort of eight children with isolated dystonia assessed by blinded video rating [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/long-term-results-of-pallidal-deep-brain-stimulation-in-a-cohort-of-eight-children-with-isolated-dystonia-assessed-by-blinded-video-rating/. Accessed November 21, 2024.« Back to 2016 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/long-term-results-of-pallidal-deep-brain-stimulation-in-a-cohort-of-eight-children-with-isolated-dystonia-assessed-by-blinded-video-rating/