Session Information
Date: Saturday, October 6, 2018
Session Title: Pediatric Movement Disorders
Session Time: 1:45pm-3:15pm
Location: Hall 3FG
Objective: This study is to review our single-center experience with deep brain stimulation (DBS) in pediatric patients with dystonia.
Background: Deep brain stimulation (DBS) is an established therapy option for movement disorders. Knowledge about the indication and efficacy in children with heterogeneous genetic and acquired dystonia is limited.
Methods: Single-center data from all pediatric patients treated with DBS before the age 18 was assessed retro- and prospectively. Etiology, surgical and stimulation parameters as well as side effects were documented. Outcome was assessed using function specific scores, quality of life questionnaires, established movement disorder rating scales and self-rating.
Results: Since 2008, 8 children (mean age at surgery 13.1 years, range 7-17 years) underwent DBS implantation at the Medical University Hospital Vienna. Two patients were diagnosed with genetic dystonia (TOR1A, KMT2B), one with Lesch-Nyhan disease, and five with acquired dystonia. All patients underwent bilateral stereotactic surgery (globus pallidus internus) under general anesthesia. Electrode revision due to displacement and intermittent hardware removal after infection was necessary in one patient each. Observation periods range from 1 to 10 years. Results of standardized assessments revealed the greatest improvements in patients with genetic dystonia but heterogeneous outcomes in acquired dystonia.
Conclusions: Our single-center experience adds to the growing evidence that DBS is a safe therapy option in pediatric patients. Patients with both well-described (TOR1A) and only recently discovered underlying genetic mutations (KMT2B) demonstrate remarkable improvements after DBS. However, within the more prevalent group of children with acquired dystonia, robust prognostic factors are still lacking. Established dystonia rating scales designed for adults do not reflect parental subjective assessment of functional outcome.
To cite this abstract in AMA style:
M. Freilinger, A. Zeitlberger, S. Siegert, R. Seidl, F. Alesch, G. Kranz, K. Novak. Deep brain stimulation matters in pediatric neurology – single-center experience [abstract]. Mov Disord. 2018; 33 (suppl 2). https://www.mdsabstracts.org/abstract/deep-brain-stimulation-matters-in-pediatric-neurology-single-center-experience/. Accessed June 14, 2025.« Back to 2018 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/deep-brain-stimulation-matters-in-pediatric-neurology-single-center-experience/