Background: Status dystonicus (SD) is a life-threatening complication in which episodes of dystonic movements become increasingly frequent and severe, requiring urgent hospital admission, and can lead to respiratory, metabolic, and bulbar complications. Pharmacologic treatment has been the mainstay management for this complication; however, many refractory patients will still require further treatment. Deep brain stimulation (DBS) is an established therapeutic strategy that has been used for dystonia, and now it has been proposed to be used for SD.
Methods: In this case series, we describe our experience with early DBS placement in 5 patients with SD to control symptoms that are refractory to pharmacologic therapy. In addition, we present a literature review of this therapy in the treatment of SD.
Results: Before discharge, symptomatic relief (decrease of dystonic movements and resolution of abnormal postures) was evidenced in all patients with a median of 3 days (interquartile range, 1-7) after surgery was performed. A follow-up Unified Dystonia Rating Scale score and Burke-Fahn-Marsden rating scale motor subscale score, at 6 months after hospital discharge with values being inferior to 20 and 30, respectively, for all cases. None of the patients had a recurrence of SD in the last follow-up period.
Conclusions: DBS surgery is a suitable, versatile, reversible and adequate therapy in the treatment of SD that is refractory to initial pharmacologic treatment.
Keywords: Deep brain stimulation; Dystonic crisis; Dystonic storm; Life-threatening dystonia; Severe dystonia; Status dystonicus.
Copyright © 2018 Elsevier Inc. All rights reserved.