Tolerance of early pallidal stimulation in pediatric generalized dystonia

Report of 2 cases

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The authors report on 2 cases of pediatric generalized dystonia with a DYT1 mutation; the patients, an 11-year-old girl and a 9-year-old boy, underwent chronic, pallidal deep brain stimulation (DBS) of the globus pallidus internus (GPi). The dystonic postures in both cases showed dramatic improvements with pallidal DBS, but each patient's symptoms gradually recurred within a year, irrespective of exhaustive readjustments of the stimulation settings. After the recurrence of the dystonic symptoms, the DBS leads were replaced within the GPi in one patient (Case 1) and additional DBS leads were implanted into the bilateral subthalamic nuclei in the other patient (Case 2). Neither measure produced any further clinical benefit, and the patient in Case 2 died of status dystonicus 2 days after reoperation. These findings suggest that early pallidal DBS for pediatric dystonia is indeed effective, although there are some cases in which its therapeutic effect is lost. One possible reason may be the ability of the preadolescent brain to tolerate chronic electrical stimuli during the active maturation process.

Abbreviations used in this paper:BFMDRS = Burke-Fahn-Marsden Dystonia Rating Scale; DBS = deep brain stimulation; GPi = globus pallidus internus; IPG = implantable pulse generator.

Article Information

Address correspondence to: Yasushi Miyagi, M.D., Ph.D., Department of Stereotactic and Functional Neurosurgery, Kaizuka Hospital, 7-7-27 Hakozaki, Higashi-ku, Fukuoka 812-8582, Japan. email:

Please include this information when citing this paper: published online September 13, 2013; DOI: 10.3171/2013.8.PEDS12578.

© AANS, except where prohibited by US copyright law.



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    Case 1. Axial (A) and coronal (B) MR images showing the location (arrows) of bilateral pallidal electrodes. Line graph (C) demonstrating the serial changes of the motor score of Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS-M; values above the graph). Arrows indicate the surgeries: R = initial pallidal DBS on the right; rp = lateral replacement of the pallidal lead; L = addition of the second pallidal DBS on the left; Lrp = lateral replacement of the pallidal lead; R/L = renewal of the bilateral pallidal leads and extension cables due to lead fracture.

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    Case 2. Axial (A) and coronal (B) MR images showing the location (arrows) of bilateral pallidal electrodes and subthalamic electrodes. Line graph (C) demonstrating the serial changes of the motor score of the BFMDRS-M (values above the graph). Arrows indicate the surgeries (far left arrows = first pallidal DBS; STN = addition of subthalamic DBS). Open circle and solid line indicate the course of the patient in Case 2; closed circle and dotted line indicate the course of the patient's father.



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