Deep Brain Stimulation for Refractory Status Dystonicus in Children: Multicenter Case Series and Systematic Review

Abstract

ObjectiveWe sought to better understand the workflow, outcomes, and complications of deep brain stimulation (DBS) for pediatric status dystonicus (SD). We present a systematic review, alongside a multi‐center case series of pediatric patients with SD treated with DBS.MethodsWe collected individual data regarding treatment, stimulation parameters, and dystonia severity for a multi‐center case series (n=8) and all previously published cases (n=77). Data for case series was used to create probabilistic voxel‐wise maps of stimulated tissue associated with dystonia improvement.ResultsIn our institutional series, DBS was implanted a mean of 25 days post‐SD onset. Programming began a mean of 1.6 days after surgery. All eight patients in our case series and 73/74 reported patients in the systematic review had resolution of their SD with DBS, most within 2‐4 weeks of surgery. Mean follow‐up for patients in the case series was 16 months. DBS target for all patients in the case series and 68/77 in our systematic review was the globus pallidus pars interna (GPi). In our case series, stimulation of the posterior‐ventrolateral GPi was associated with improved dystonia. Mean dystonia improvement was 32% and 51% in our institutional series and systematic review, respectively. Mortality was 4% in the review, which is lower than reported for treatment with pharmacotherapy alone (10‐12.5%).InterpretationDBS is a feasible intervention with potential to reverse refractory pediatric SD and improve survival. More work is needed to increase awareness of DBS in this setting, so that it can be implemented in a timely manner.This article is protected by copyright. All rights reserved.