Treatment of status dystonicus (dystonic storm)

Abstract

Status dystonicus is an acute potentially fatal movement disorder that occasionally develops in the course of primary and secondary dystonic syndromes and is characterized by increasingly frequent and severe episodes of generalized involuntary sustained or intermittent muscle contractions resulting in abnormal postures or repetitive twisting movements. It may be complicated by bulbar and respiratory complications and rhabdomyolysis which may lead to acute renal failure. Early recognition of the signs and symptoms of this condition and its complications is needed for successful management. Stabilizing measures often entail tracheal intubation and mechanical ventilation along with powerful sedative agents given in the intensive care unit. Frequently, a combination of dystonia-specific medications can be used to alleviate the dystonia. If dystonia persists despite treatment with these drugs, then intrathecal baclofen or deep brain stimulation should be considered.