Clinical presentation of new onset refractory status epilepticus in children (the pSERG cohort)
Author
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Sculier, Claudine
Author
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Aguilar, Cristina Barcia
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Gaspard, Nicolas
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Gainza Lein, Marina Andrea
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Sánchez Fernández, Iván
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Amengual Gual, Marta
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Anderson, Anne
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Arya, Ravindra
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Burrows, Brian T.
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Brenton, James N.
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Carpenter, Jessica L.
Author
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Chapman, Kevin E.
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Clark, Justice
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Gaillard, William D.
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Glauser, Tracy A.
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Goldstein, Joshua L.
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Goodkin, Howard P.
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Gorman, Mark
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Lai, Yi-Chen
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McDonough, Tiffani L.
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Mikati, Mohamad A.
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Nayak, Anuranjita
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Peariso, Katrina
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Riviello, James
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Rusie, Allison
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Sperberg, Katherine
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Stredny, Coral M.
Author
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Tasker, Robert C.
Author
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Tchapyjnikov, Dmitry
Author
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Vásquez, Alejandra
Author
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Wainwright, Mark S.
Author
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Wilfong, Angus A.
Author
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Williams, Korwyn
Author
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Loddenkemper, Tobias
Admission date
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2022-03-21T19:30:08Z
Available date
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2022-03-21T19:30:08Z
Publication date
dc.date.issued
2021
Cita de ítem
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Epilepsia. 2021;62:1629–1642.
es_ES
Identifier
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10.1111/epi.16950
Identifier
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https://repositorio.uchile.cl/handle/2250/184294
Abstract
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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.
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Patrocinador
dc.description.sponsorship
Epilepsy Research Fund
Epilepsy Foundation of America EF-213583
American Epilepsy Society/Epilepsy Foundation of America Infrastructure Award
Pediatric Epilepsy Research Foundation
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Lenguage
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en
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Publisher
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Wiley
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Type of license
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Attribution-NonCommercial-NoDerivs 3.0 United States