C.4 Understanding the role of deep brain stimulation for Refractory Status Dystonicus in children: case series and systematic review

Abstract

Background: Status dystonicus (SD) is a life-threatening form of dystonia with limited treatments available. We sought to better understand the processes, outcomes, and complications of deep brain stimulation (DBS) for pediatric SD through a systematic review alongside an institutional case series. Methods: Data regarding treatment, stimulation parameters, dystonia severity and outcomes was collected for the case series (n=7) and systematic review (n=70, conducted in accordance with PRISMA guidelines). This was analysed descriptively (rates, outcome measures). For the case series we created probabilistic voxel-wise maps for improvement in dystonia based on brain region stimulated. Results: All patients in our case series and > 95% of patients in the systematic review had resolution of SD with DBS, typically within 2-4 weeks. Most patients in the review (84%) and all patients in the case series had DBS implanted to the globus pallidus internus. In terms of dystonia severity scores, there was a mean improvement of 25% (case series) or 49% (systematic review). Reported mortality was 4% in the systematic review. Conclusions: DBS for pediatric SD is feasible and safe. It allows for increased survival as well as quality of life - however risks still exist. More work is needed to determine timing, eligibility, and stimulation parameters.