Pallidal Stimulation and Status Dystonicus in DYT1 Primary Generalized Dystonia

Keywords: dystonia, globus pallidus, deep brain stimulation, outcome, movement disorder

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     Status Dystonicus, also known as dystonic storm, is a rare complication of primary and secondary dystonias characterized by acute worsening of dystonic movements, and can progress to a life-threatening emergency.
     There have been less than 50 patients reported in the literature, with widely varying causes, manifestations, and treatments with varying efficacy. Recently pallidal deep brain stimulation (DBS) has emerged as a potentially effective form of therapy.
     We report 5 cases of status dystonicus in children with DYT1-positive dystonia treated acutely with pallidal DBS.
     All cases were performed by senior author in our tertiary care center with a movement disorders team experienced in the neurosurgical and neurologic care of patients with pallidal stimulation for dystonia.
     Of the five reported cases, two patients developed severe exacerbation of their dystonia due to underlying medical illnesses, one case was exacerbated due to severe infection, while two others developed their symptoms following complications involving previously implanted DBS systems. All patients were implanted under sedation while in a state of status dystonicus. All patients subsequently recovered after various lengths of stay with implantation, revision or initiation stimulation.
     This is a retrospective study.
     Status dystonicus, or dystonic storm, is a rare and potentially life-threatening complication of many dystonic syndromes.
     To our knowledge, this is the first and largest case-series reporting the use of DBS as an acute treatment for dystonic storm. Our experiences with using DBS as a means of treating acute dystonic storm crises presents a new effective treatment for this potentially life threatening emergency.


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