A retrospective study of 92 children with new-onset refractory status epilepticus

Abstract

Little is known of the etiology, course, and treatment of new-onset refractory status epilepticus (NORSE) in children. Here we identified etiologies, electroencephalography (EEG) characteristics, and neuroimaging findings among pediatric patients with NORSE and among two patient subgroups, febrile infection-related epilepsy syndrome (FIRES) group and non-FIRES group. We also examined treatments and risk factors related to poor prognosis. Ninety-two children with NORSE were identified in Children's Hospital of Chongqing Medical University between January 1, 2010 and September 1, 2020. The end date was chosen to guarantee at least a 6-month follow-up. Our results indicated that patients with FIRES account for 90% of pediatric patients with NORSE. The clinical, EEG, and neuroimaging results and prognosis were not significantly different between the FIRES group and non-FIRES group of individuals. 68.5% of our patients had unknown etiology, and viral etiology was the most common identified cause (26.1%). Electroencephalography might have a certain diagnostic value for NORSE. A gradual increase in seizure burden was obvious from the onset of disease, and continuous or recurrent ictal discharge lasting ≥ 30 min was quite common in our study. The mortality was 22.8% in our study. Among the 71 surviving patients, the outcome at discharge was poor but improved during follow-up, and 68.5% had good or fair outcomes at their last follow-up. A poor outcome was observed in 39 of 92 cases (42%), with 43.9% and 30% of individuals in the FIRES group and non-FIRES group, respectively, having a poor outcome. The presence of super refractory status epilepticus (SRSE), electrographic seizures and nonconvulsive status epilepticus (NCSE), and diffuse cortical edema and multifocal abnormality may be related to a poor prognosis. Our analysis did not indicate that prognosis was directly related to etiology or treatment. Management of NORSE is challenging, and the role of immunotherapy warrants further investigation.