Review articleHistorical developments in children's deep brain stimulation

- Childhood onset dystonia is frequently progressive, with worsening towards generalization and consequent disability.
- GPi DBS is efficient in isolated dystonia, subcortical myoclonus, dystonic tremor and tics; STN and motor thalamic nuclei are under exploration.
- In acquired dystonia, neurometabolic and degenerative disorders, DBS role deserves exploration to confirm as an efficient and lasting therapy.
- The choice of neuromodulatory DBS parameters could influence not only the initial therapeutic outcome but also its maintenance over time.
- DBS opens a new window towards a better understanding of the physiology of different movement and psychiatric disorders.

BackgroundHeterogeneous by the underlying pathobiology and clinical presentation, childhood onset dystonia is most frequently progressive, with related disability and limitations in functions of daily living. Consequently, there is an obvious need for efficient symptomatic therapies.Methods and ResultsFollowing lesional surgery to basal ganglia (BG) and thalamus, deep brain stimulation (DBS) is a more conservative and adjustable intervention to and validated for internal segment of the globus pallidus (GPi), highly efficient in treating isolated “primary” dystonia and associated symptoms such as subcortical myoclonus. The role of DBS in acquired, neurometabolic and degenerative disorders with dystonia deserves further exploration to confirm as an efficient and lasting therapy. However, the pathobiological background with distribution of the sequellae over the central nervous system and related clinical features, will limit DBS efficacy in these conditions. Cumulative arguments propose DBS in severe life threatening dystonic conditions called status dystonicus as first line therapy, irrespective of the underlying cause.There are no currently available validated selection criteria for DBS in pediatric dystonia. Concurrent targets such as subthalamic nucleus (STN) and several motor nuclei of the thalamus are under exploration and only little information is available in children. DBS programming in paediatric population was adopted from experience in adults. The choice of neuromodulatory DBS parameters could influence not only the initial therapeutic outcome of dystonic symptoms but also its maintenance over time and potentially the occurrence of DBS related side effects.ConclusionDBS allows efficient symptomatic treatment of severe dystonia in children and advances pathophysiological knowledge about local and distributed abnormal neural activity over the motor cortical-subcortical networks in dystonia and other movement disorders.