Historical Developments in Children’s Deep Brain Stimulation

Abstract Since the initial description of dystonia as a severe and potentially life threatening condition with limited potential for spontaneous remission, there is an obvious need for efficient symptomatic therapies. Heterogeneous by the underlying pathobiology and clinical presentation, childhood onset dystonia is most frequently progressive, with worsening towards generalization and consequent disability and limitations in functions of daily living. Following lesional surgery to basal ganglia (BG) and thalamus for treating dystonia, deep brain stimulation (DBS) is a more conservative and adjustable symptomatic therapeutic intervention to and validated for internal segment of the globus pallidus (GPi). DBS is highly efficient in treating isolated “primary” dystonia in children. Furthermore, associated symptoms such as subcortical myoclonus encountered in combined dystonia can be suppressed by DBS. While GPi DBS efficacy has been validated by prospective controlled studies in dystonia in adults, 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. Just as DBS indicated for primary pediatric dystonia, need exist to treat acquired and combined dystonia, with symptoms as severe as in isolated forms, but associated with more complex neurological symptoms. The pathobiological background, with distribution of the sequellae over the central nervous system and related clinical features, will limit DBS efficacy in these conditions. There are no currently available validated selection criteria for DBS in pediatric dystonia. However, recent efforts and technological progress in neuroimaging, neurophysiology and more accurate and quantified clinical assessment, together with functional and quality of life measures, provide an improved “package” for building up the baseline “frame” benefiting from DBS. Several acquired dystonia syndromes are reported to benefit from DBS in selected cases. The role of DBS as symptomatic treatment in neurometabolic and degenerative disorders with dystonia in children deserves further attention and exploration to confirm as an efficient and lasting therapy. However, there are cumulative arguments to propose DBS in severe life threatening dystonic conditions called status dystonicus as first line therapy, since it is most efficient in these severe conditions, irrespective of the underlying cause. One aspect that deserves to