PMID- 34794014 OWN - NLM STAT- MEDLINE DCOM- 20211210 LR - 20211214 IS - 1525-5069 (Electronic) IS - 1525-5050 (Linking) VI - 125 DP - 2021 Dec TI - A retrospective study of 92 children with new-onset refractory status epilepticus. PG - 108413 LID - S1525-5050(21)00674-0 [pii] LID - 10.1016/j.yebeh.2021.108413 [doi] AB - 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. CI - Copyright (c) 2021 Elsevier Inc. All rights reserved. FAU - Wu, Jinfeng AU - Wu J AD - Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, China. FAU - Lan, Xinghui AU - Lan X AD - Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, China. FAU - Yan, Lisi AU - Yan L AD - Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, China. FAU - Hu, Yue AU - Hu Y AD - Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, China. FAU - Hong, Siqi AU - Hong S AD - Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, China. FAU - Jiang, Li AU - Jiang L AD - Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, China. FAU - Chen, Jin AU - Chen J AD - Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, China. Electronic address: chenjinsuper@hotmail.com. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20211115 PL - United States TA - Epilepsy Behav JT - Epilepsy & behavior : E&B JID - 100892858 SB - IM MH - Child MH - *Drug Resistant Epilepsy/diagnosis/epidemiology MH - Electroencephalography MH - Humans MH - Retrospective Studies MH - Seizures MH - *Status Epilepticus/diagnosis/epidemiology/etiology OTO - NOTNLM OT - Children OT - Febrile infection-related epilepsy syndrome OT - New-onset refractory status epilepticus EDAT- 2021/11/19 06:00 MHDA- 2021/12/15 06:00 CRDT- 2021/11/18 20:17 PHST- 2021/07/28 00:00 [received] PHST- 2021/10/25 00:00 [revised] PHST- 2021/10/26 00:00 [accepted] PHST- 2021/11/19 06:00 [pubmed] PHST- 2021/12/15 06:00 [medline] PHST- 2021/11/18 20:17 [entrez] AID - S1525-5050(21)00674-0 [pii] AID - 10.1016/j.yebeh.2021.108413 [doi] PST - ppublish SO - Epilepsy Behav. 2021 Dec;125:108413. doi: 10.1016/j.yebeh.2021.108413. Epub 2021 Nov 15.