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Clinical presentation of new onset refractory status epilepticus in children (the pSERG cohort)
Author(s) -
Sculier Claudine,
Barcia Aguilar Cristina,
Gaspard Nicolas,
GaínzaLein Marina,
Sánchez Fernández Iván,
AmengualGual 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 YiChen,
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
Publication year - 2021
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/epi.16950
Subject(s) - etiology , medicine , pediatrics , cohort , refractory (planetary science) , epilepsy syndromes , epilepsy , status epilepticus , psychiatry , biology , astrobiology
Objective We 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). Methods Patients 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. Results Of 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. Significance Among 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.