Treatment options in pediatric super-refractory status epilepticus

Background: Super-refractory status epilepticus (SRSE) is a seizure that continues >24 h after anesthesia, or recurs on the reduction of anesthesia. SRSE is extremely difficult-to-control and associated with poor outcome. To date, optimal therapy and outcome data in children is limited. Objective: To assess etiology, treatment options and outcome in pediatric SRSE patients. Method: We reviewed medical records of children <15 years old with SRSE during 2007-2017 at King Chulalongkorn Memorial Hospital. Demographic data, etiology, treatment, complications and discharge outcome were recorded. Results: Seventeen patients, aged 1 month-13 years were included. The leading etiology was immune-mediated encephalitis (29.4%) and epilepsy (29.4%). The most common anesthetic agents were midazolam (94.1%) and propofol (52.9%) with the average maximal dose of 1.3 and 6.9 mg/kg/h respectively. Other treatments included immunological therapy (76.5%), ketogenic diet (76.5%), pyridoxine/pyridoxal-5-phosphate (70.5%). The most common complications were hypotension (61.5%), drug hypersensitivity (32.5%). Median length of anesthetic and intensive care were 9 and 23 days. The mortality rate was 17.6%, and 2 of 3 febrile infection-related epilepsy syndrome cases died. At discharge, all survivors were seizure free. Conclusion: The majority of pediatric SRSE does not have epilepsy and the etiology is various. Treatment should expand from antiepileptic drugs to other modalities targeting different possible mechanisms such as immunomodulation or specific metabolic treatment. Multiple anesthetic drugs could be tolerated with close monitoring. Ketogenic diet, via enteral or parenteral route, could be considered early if requiring multiple anesthetic drugs. Initial outcome in children is relatively better than in adults. (C) 2018 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.