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Four children with dystonic storm are shown in this video segment. The first video shows a pediatric case of status dystonic triggered by DBS surgery in a 9-year-old boy with generalized hyperkinetic movements possibly caused by an ADCY-5 mutation. Three patients with DYT-1 dystonia and dystonic storm are shown next. The first young man developed acute marked worsening of his dystonia, with severe generalized spasms involving all four limbs that required hospitalization in the intensive care unit. Despite numerous oral and intravenous medications, his dystonic spasms could not be controlled, and he eventually underwent placement of a baclofen pump (a standard procedure in the late 1990s, but not one that is used today for this entity).
