Neuromodulation in Childhood Onset Dystonia: Evolving Role of Deep Brain Stimulation

This review will provide an update on the current status of deep brain stimulation in pediatric onset dystonias. Dystonia is a complex movement disorder that may occur in isolation or in combination with other abnormalities of tone and posture. The dystonias represent a heterogeneous group of movement disorders that can be progressive, painful, and severely disabling. Genetic and extrinsic factors may influence the treatment response. Pharmacologic treatment often has limited effectiveness and unacceptable, unwanted effects. Neuromodulation by deep brain stimulation (DBS) is a targeted therapy that continues to evolve as a treatment option for children with medically refractory dystonia due to a variety of etiologies. We share some insights from our surgical experience with 124 children with dystonia treated by DBS and review the current literature on the use of DBS in pediatric onset dystonia. Advances in surgical options make the surgery more tolerable for young children. Secondary dystonia, in particular cerebral palsy, is more common than primary genetic etiologies. Both may respond to DBS, which may be considered once medical management has failed. Data sharing through registry platforms such as PEDiDBS is a vital component for expanding our knowledge base.