gms | German Medical Science

58. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e. V. (DGNC)

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

26. bis 29.04.2007, Leipzig

Early chronic GPI stimulation in paediatric patients suffering from intractable primary dystonia

Frühzeitige tiefe Hirnstimulation im GPI bei pädiatrischen Patienten mit therapierefraktärer primärer Dystonie

Meeting Abstract

  • corresponding author J.H. Mehrkens - Neurochirurgische Universitätsklinik, Ludwig-Maximilians-Universität München
  • K. Bötzel - Neurologische Universitätsklinik, Ludwig-Maximilians-Universität München
  • F. Heinen - Universitätskinderklinik, Ludwig-Maximilians-Universität München
  • S. Berweck - Universitätskinderklinik, Ludwig-Maximilians-Universität München
  • U. Steude - Neurochirurgische Universitätsklinik, Ludwig-Maximilians-Universität München

Deutsche Gesellschaft für Neurochirurgie. 58. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC). Leipzig, 26.-29.04.2007. Düsseldorf: German Medical Science GMS Publishing House; 2007. DocSO.06.06

The electronic version of this article is the complete one and can be found online at:

Published: April 11, 2007

© 2007 Mehrkens et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.



Objective: GPI (globus pallidus internus) deep brain stimulation (DBS) offers a very effective stereotactic neurosurgical therapy for medically intractable dystonia. Although generalized dystonia is presenting mainly at young age and might be severely disabling, GPI-DBS is still usually not applied in the early course of the disease. We here present the immediate post-operative results and clinical follow for 5 paediatric patients undergoing GPI-DBS.

Methods: All patients (mean age 13 years (range 8-16 years), 3 female/2 male, 2 DYT1-positive) were suffering from intractable generalized dystonia. Electrode-implantation (DBS 3389, Medtronic) was performed under propofol anaesthesia with MRI-guided stereotaxy using a modified Leksel/Lerch system. The lowest possible electrode was chosen for stimulation and intensity was set relatively high from the beginning (mean 80µA). After one week of successful stimulation by an external test stimulation system, permanent implantation of the stimulation system (Soletra/Kinetra, Medtronic) was performed. Electrode position was verified by MRI and clinical outcome was assessed using the Burke-Fahn-Marsden (BFM) motor and disability scores (mean follow-up 30 months, range 12-60 months).

Results: All patients experienced a significant improvement of symptoms already during the first week. After 3 months, the BFM scores had improved in the range from 75% to 82% compared to preoperatively. After 12 months, relief of symptoms was unchanged compared to the 3-month status in 2 patients and further improved in 3 patients. The 2 patients with long-term follow-up (60 months) showed a stable relief of symptoms. Local infection occurred in one patient, making electrode-explantation necessary. Re-implantation was performed three months later without further complications and stable effectiveness. No other therapy-related morbidity was observed.

Conclusions: GPI deep brain stimulation offers a very effective and safe therapy in paediatric patients suffering from primary dystonia. Stable relief of symptoms can be achieved. Early neurosurgical intervention is crucial to prevent irreversible impairment of motor function.