Deep brain stimulation and dystonia: About 10 new pediatric cases and a new DBS center in Paris

Abstract

Objective: Deep Brain Stimulation (DBS) is a surgical technique used for dystonic patients during which a continuous electrical stimulation is delivered to the Globus Pallidum. Technical improvements render this procedure possible for young children. However, predictive factors for good responders remain poorly understood. We opened a new DBS pediatric center in 2015. We present our first results and the organization of our multidisciplinary care system. Methods: We report a series of 10 children seen in consultation for DBS during 2015–2016. In the absence of radiological or etiologic contraindications, the files are reviewed and the indications for implantation are validated in multidisciplinary consultation (neuropediatricians, neurologists, neurosurgeons, rehabilitation physicians). Once included, children are evaluated pre- and post-operatively by the “dystonia referents” team within the hospital (psychologist, physiotherapist, nurse, anesthetist, resuscitator, ENT, photographer for documentation of symptoms) conjointly with the neuropediatrician and neurosurgeon. Results: 5 children were implanted (2 DYT1 mutations, 1 PKAN, 1 severe dyskinetic dystonia, 1 generalized dystonia), 3 were finally excluded and 2 refused intervention. All implanted patients improved (video), between 30 to 95% (patient DYT1). The decreased dyskinesias has also uncovered learning abilities in the dyskinetic patient. Conclusion: DBS of the GPi has a well-known efficacy in DYT1, however recent studies as ours show that other primary or secondary dystonias may benefit as well from such interventions. Implantation in young children, however, poses a problem concerning the cumbersomeness long-term follow-up and need for regular re- interventions. The indications for implantation require in our opinion a multidisciplinary decision and to obtain the perfect adhesion of the family. A protocoled and rigorous follow-up of this population will improve both the selection criteria and suggest predictive parameters for better responders.