Movement Disorder Emergencies

Part of the series Current Clinical Neurology pp 125-135


Dystonic Storm

  • Melissa J. NirenbergAffiliated withDivision of Movement Disorders, New York University School of Medicine Email author 
  • , Blair FordAffiliated withDepartment of Neurology, Neurological Institute, Columbia University Medical Center/New York Presbyterian Hospital

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In rare cases, generalized dystonia can rapidly progress to cause severe, continuous forceful contractions. This phenomenon—referred to as “dystonic storm” or “status dystonicus”—is a severe, life-threatening crisis that requires urgent evaluation and treatment. Dystonic storm can occur in idiopathic dystonia, or in symptomatic dystonia due to trauma, encephalitis, static encephalopathy, acute neuroleptic exposure, or neurodegenerative disorders. The differential diagnosis of dystonic storm includes bacterial meningitis, neuroleptic malignant syndrome, serotonin syndrome, malignant hyperthermia, and intrathecal baclofen withdrawal. Treatments for dystonic storm are empiric, and can be divided into three categories: supportive care, temporizing measures, and dystonia-specific treatments. Supportive care includes admission to an intensive care unit, mechanical ventilation, treatment with intravenous fluids and antipyretics, and monitoring of creatine phosphokinase, renal function, and urine output. Potential triggers (such as infection or medications) should also be identified and treated. Temporizing measures include the use of sedative-hypnotics, general anesthetics, or non-depolarizing paralytic agents. Dystonia-specific therapies should begin with catecholamine-depleting agents, anticholinergics, and/or dopamine receptor blocking agents. Other medications that may be effective include baclofen and benzodiazepines. If oral dystonia therapies are ineffective, then interventions such as an intrathecal baclofen pump or deep brain stimulation of the globus pallidus interna should be considered.