Status Dystonicus: A Rare and Underdiagnosed Complication of Dystonia

Abstract

Dystonia is characterized by sustained muscle contractions which produce repetitive twisting movements or abnormal postures. If it is associated with intense frequent episodes refractory to standard drug therapy and requires urgent hospital management, it is known as status dystonicus (SD). Pediatric SD remains underdiagnosed and is a potentially life-threatening crisis. SD is very painful and uncomfortable to the patient and very much distressing to the care givers. Complications of SD include bulbar weakness compromising the upper airway, pulmonary aspiration, respiratory failure, metabolic derangements such as rhabdomyolysis, myoglobinuria, dehydration, acute renal failure and hyperpyrexia. The efficacy of medical management of SD is only 10% and mortality remains at about 10%. We report on 2 dystonic cerebral palsy children who presented to us with SD: one in respiratory failure who needed immediate intubation and a second case who was under diagnosed at admission, later diagnosed during an intensive care unit stay who partially responded to midazolam infusion and polypharmacotheraphy.