Long-term neuropsychological outcomes in children with febrile infection-related epilepsy syndrome (FIRES) treated with anakinra.

Autor:	Shrestha A; University of Colorado School of Medicine, Aurora, CO, United States., Wood EL; Department of Pediatrics, Section of Neurology, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO, United States., Berrios-Siervo G; Department of Pediatrics, Section of Neurology, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO, United States.; Department of Pediatrics, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO, United States., Stredny CM; Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Boston, MA, United States.; Program in Neuroimmunology, Department of Neurology, Boston Children's Hospital, Boston, MA, United States., Boyer K; Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Boston, MA, United States., Vega C; Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Boston, MA, United States., Nangia S; Department of Child Neurology, Weill Cornell Medicine, New York-Presbyterian Hospital, New York, NY, United States., Muscal E; Department of Pediatrics and Child Neurology (Co-appointment), Baylor College of Medicine, Houston, TX, United States., Eschbach K; Department of Pediatrics, Section of Neurology, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, CO, United States.
Jazyk:	angličtina
Zdroj:	Frontiers in neurology [Front Neurol] 2023 Mar 08; Vol. 14, pp. 1100551. Date of Electronic Publication: 2023 Mar 08 (Print Publication: 2023).
DOI:	10.3389/fneur.2023.1100551
Abstrakt:	Background: Febrile-infection related epilepsy syndrome (FIRES) is a rare epilepsy syndrome in which a previously healthy individual develops refractory status epilepticus in the setting of a preceding febrile illness. There are limited data regarding detailed long-term outcomes. This study aims to describe the long-term neuropsychological outcomes in a series of pediatric patients with FIRES. Methods: This is a retrospective multi-center case series of pediatric patients with a diagnosis of FIRES treated acutely with anakinra who had neuropsychological testing at least 12 months after status epilepticus onset. Each patient underwent comprehensive neuropsychological evaluation as part of routine clinical care. Additional data collection included the acute seizure presentation, medication exposures, and outcomes. Results: There were six patients identified with a median age of 11.08 years (IQR: 8.19-11.23) at status epilepticus onset. Anakinra initiation was a median of 11 days (IQR: 9.25-13.50) after hospital admission. All patients had ongoing seizures and none of the patients returned to baseline cognitive function with a median follow-up of 40 months (IQR 35-51). Of the five patients with serial full-scale IQ testing, three demonstrated a decline in scores over time. Testing results revealed a diffuse pattern of deficits across domains and all patients required special education and/or accommodations for academic learning. Conclusions: Despite treatment with anakinra, neuropsychological outcomes in this series of pediatric patients with FIRES demonstrated ongoing diffuse neurocognitive impairment. Future research will need to explore the predictors of long-term neurocognitive outcomes in patients with FIRES and to evaluate if acute treatment interventions improve these outcomes. Competing Interests: EM serves on the advisory board for Sobi. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. (Copyright © 2023 Shrestha, Wood, Berrios-Siervo, Stredny, Boyer, Vega, Nangia, Muscal and Eschbach.)
Databáze:	MEDLINE
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