Clinical presentation of new onset refractory status epilepticus in children (the pSERG cohort).

Content Provider	Europe PMC
Author	Sculier, Claudine Barcia Aguilar, Cristina Gaspard, Nicolas Gaínza‐Lein, Marina Sánchez Fernández, Iván Amengual‐Gual, Marta Anderson, Anne Arya, Ravindra Burrows, Brian T. Brenton, James N. Carpenter, Jessica L. Chapman, Kevin E. Clark, Justice Gaillard, William D. Glauser, Tracy A. Goldstein, Joshua L. Goodkin, Howard P. Gorman, Mark Lai, Yi‐Chen McDonough, Tiffani L. Mikati, Mohamad A. Nayak, Anuranjita Peariso, Katrina Riviello, James Rusie, Allison Sperberg, Katherine Stredny, Coral M. Tasker, Robert C. Tchapyjnikov, Dmitry Vasquez, Alejandra Wainwright, Mark S. Wilfong, Angus A. Williams, Korwyn Loddenkemper, Tobias pSERG
Abstract	AbstractObjectiveWe aimed to characterize the clinical profile and outcomes of new onset refractory status epilepticus (NORSE) in children, and investigated the relationship between fever onset and status epilepticus (SE).MethodsPatients with refractory SE (RSE) between June 1, 2011 and October 1, 2016 were prospectively enrolled in the pSERG (Pediatric Status Epilepticus Research Group) cohort. Cases meeting the definition of NORSE were classified as "NORSE of known etiology" or "NORSE of unknown etiology." Subgroup analysis of NORSE of unknown etiology was completed based on the presence and time of fever occurrence relative to RSE onset: fever at onset (≤24 h), previous fever (2 weeks–24 h), and without fever.ResultsOf 279 patients with RSE, 46 patients met the criteria for NORSE. The median age was 2.4 years, and 25 (54%) were female. Forty (87%) patients had NORSE of unknown etiology. Nineteen (48%) presented with fever at SE onset, 16 (40%) had a previous fever, and five (12%) had no fever. The patients with preceding fever had more prolonged SE and worse outcomes, and 25% recovered baseline neurological function. The patients with fever at onset were younger and had shorter SE episodes, and 89% recovered baseline function.SignificanceAmong pediatric patients with RSE, 16% met diagnostic criteria for NORSE, including the subcategory of febrile infection‐related epilepsy syndrome (FIRES). Pediatric NORSE cases may also overlap with refractory febrile SE (FSE). FIRES occurs more frequently in older children, the course is usually prolonged, and outcomes are worse, as compared to refractory FSE. Fever occurring more than 24 h before the onset of seizures differentiates a subgroup of NORSE patients with distinctive clinical characteristics and worse outcomes.
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Page Count	14
ISSN	00139580
Journal	Epilepsia
Volume Number	62
DOI	10.1111/epi.16950
PubMed Central reference number	PMC8362203
Issue Number	7
PubMed reference number	34091885
e-ISSN	15281167
Language	English
Publisher	John Wiley and Sons Inc.
Publisher Date	2021-06-06
Publisher Place	Hoboken
Access Restriction	Open
Rights License	This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes. © 2021 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.
Subject Keyword	clinical neurology epilepsy febrile infection‐related epilepsy syndrome new onset refractory status epilepticus pediatric refractory status epilepticus status epilepticus
Content Type	Text
Resource Type	Article
Subject	Neurology Neurology (clinical)