Deep Brain Stimulation in Children with Dystonia: Experience from a Tertiary Care Center (S28.006)

Abstract

Objective: To investigate the efficacy and safety of deep brain stimulation (DBS) of globus pallidus internus (GPi) in children with dystonia. Background Deep brain stimulation (DBS) is an effective treatment for medically refractory dystonia. A few studies in children have shown utility of DBS in primary > secondary dystonia. More studies are needed. Design/Methods: IRB approved retrospective chart review of patients (≤21 years) with dystonia who underwent DBS of GPi. Optimal stimulation parameters and complications were recorded. Outcome measures assessed were change in the Burke-Fahn-Marsden-Dystonia-Rating-Scale and functional status post-surgery. Results: Eight patients underwent DBS for dystonia. Mean age of onset of dystonia was 7.5 ± 4.8, 7 were male. Mean age at DBS was 14.1 ± 4.6 years. Etiology of dystonia was primary in 6 [DYT-1+ (1), DYT-11 + (1), idiopathic DYT-1 negative (4)], and secondary in 2 [mitochondrial disorder (1), traumatic brain injury (1)]. Six had generalized dystonia, 1 hemidystonia, 1 cranio-cervical dystonia. All underwent GPi implantation, 1 unilateral and the rest bilateral. Mean follow-up was 6.7 ± 2.6 years, 1 lost to follow-up. Mean Burke-Fahn-Marsden-Dystonia-Rating-Scale was 62 ± 32.3 prior to surgery followed by improvement at 6 months (44± 27), 12 months (33± 24.7) and at last follow-up (31.5± 24). Mean DBS parameters at the time of maximal improvement of dystonia-rating-scale were: amplitude 3.2±0.7 volts, pulse width 170± 68 microseconds and frequency 102± 35 Hz. Three patients who were nonambulatory prior to surgery were able to ambulate without assistance after DBS. Hardware problems (electrode dislocation and breakage of cable) were observed in 2 and infection was noted in 1; no other therapy-related morbidity was observed. Conclusions: DBS of the GPi is an effective and safe therapy in pediatric patients with primary as well as selected cases of secondary dystonia. Early neurosurgical intervention seems to be crucial to prevent irreversible impairment of motor function. Disclosure: Dr. Ghosh has nothing to disclose. Dr. Machado has received personal compensation for activities with Intelect Medical, Boston scientific, ATI Pharmacology, Cardionomics and Monteris Medical as a consultant. Dr. Deogaonkar has received personal compensation for activities with Medtronic as a consultant. Dr. Deogaonkar has received (royalty or license fee or contractual rights) payments from Autonomic Technology Inc. Dr. Ghosh has received personal compensation for activities with Merz Pharma as a consultant.