gms | German Medical Science

59th Annual Meeting of the German Society of Neurosurgery (DGNC)
3rd Joint Meeting with the Italian Neurosurgical Society (SINch)

German Society of Neurosurgery (DGNC)

1 - 4 June 2008, Würzburg

Article

Send article

Deep brain stimulation of the globus pallidus internus for childhood-onset dystonia

Tiefe Hirnstimulation des Globus pallidus internus bei Kindern und Jugendlichen mit therapie-refraktärer Dystonie

Meeting Abstract

Search Medline for

  • corresponding author M.O. Pinsker - Klinik für Neurochirurgie, Universitätsklinikum Schleswig-Holstein, Campus Kiel
  • J. Volkmann - Klinik für Neurologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel
  • D. Falk - Klinik für Neurochirurgie, Universitätsklinikum Schleswig-Holstein, Campus Kiel
  • J. Herzog - Klinik für Neurologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel
  • G. Deuschl - Klinik für Neurologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel
  • H. M. Mehdorn - Klinik für Neurochirurgie, Universitätsklinikum Schleswig-Holstein, Campus Kiel

Deutsche Gesellschaft für Neurochirurgie. Società Italiana di Neurochirurgia. 59. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3. Joint Meeting mit der Italienischen Gesellschaft für Neurochirurgie (SINch). Würzburg, 01.-04.06.2008. Düsseldorf: German Medical Science GMS Publishing House; 2008. DocDI.07.06

The electronic version of this article is the complete one and can be found online at: http://www.egms.de/en/meetings/dgnc2008/08dgnc194.shtml

Published: May 30, 2008

© 2008 Pinsker et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.

Outline

Text

Objective: In prospective randomized studies deep brain stimulation (DBS) of the globus pallidus internus (Gpi) has been shown to be effective in adult patients with idiopathic generalized or segmental dystonia. Early onset idiopathic generalised dystonia is a progressive and disabling condition with loss of ambulation, speech and difficulties with feeding. We describe results of deep brain stimulation of the Gpi in three children and eight young adults with early-onset generalized dystonia.

Methods: Three children (mean age 12.3 years, range 9–14 years) and eight young adults (mean age 21.6 years, range 19–25 years) underwent bilateral DBS of the Gpi due to failure of medical treatment for generalized dystonia. Mean duration of the disease was 10.6 years (range 5–14 years) in children and 17 years (range 10–21 years) in young adults. The complete procedure was performed under general anaesthesia, including stereotactic MRI, electrophysiological mapping of the Gpi, intraoperative test stimulation to determine thresholds for stimulation-induced adverse effects, and implantation of the pulse generator.

Results: The overall clinical improvement from baseline was in mean 68.86% (range 20.39 to 98.52%) after in mean 28 months (range 6–48 months) according to the Burke-Fahn-Marsden-Dystonia-Rating-Scale (BFMDRS). In one patient postoperative MRI showed a symptomatic infarction of the Gpi with a reversible paresis and an asymptomatic small haemorrhage in the contralateral Gpi.

Conclusions: Deep brain stimulation of the Gpi is an effective treatment also for childhood-onset dystonia. Severe secondary problems, such as irreversible orthopaedic defective positions or psychosocial mental retardation can be avoided by an earlier intervention.