CASE REPORT article
Front. Pediatr.
Sec. Pediatric Critical Care
Volume 13 - 2025 | doi: 10.3389/fped.2025.1621719
This article is part of the Research TopicNew Insights into Pediatric Neurology: Neurological Disorders and Epileptic EncephalopathiesView all 15 articles
Case Report: Successful Multimodal Management of FIRES in a Pediatric Patient Using Anakinra, Ketogenic Diet and Hypothermia
Provisionally accepted
- Agostino Gemelli University Polyclinic (IRCCS), Rome, Italy
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Febrile infection–related epilepsy syndrome is a devastating refractory epileptic encephalopathy that typically occurs in school-aged, otherwise healthy, children after a brief, nonspecific, febrile illness. Herein, we discuss a case of a previously healthy 10-year-old girl with FIRES in whom high dosages of conventional and nonconventional antiepileptic drugs were ineffective in treating refractory status epilepticus and in whom intravenous corticosteroids, immunoglobulins, ketogenic diet, and induced hypothermia were adopted. We conducted an extensive workup including lumbar puncture, blood tests, continuous EEG monitoring, brain MRI, autoimmunity and infectious diseases panel to shed light on the etiology of the condition. Our patient responded to immunosuppressive therapy and ketogenic diet while her condition gradually improved up to full recovery with only mild neurocognitive sequelae. Despite patients suffering from FIRES have been described in literature, optimal management is not yet known, and prospective cohort studies are needed. This case highlights the potential benefit of a timely, multimodal therapeutic approach combining immunomodulation, metabolic support, and neuroprotection. Early administration of anakinra and initiation of a ketogenic diet may help control seizures and reduce the duration of ICU stay. Prompt diagnosis and interdisciplinary care are essential to improve outcomes in this life-threatening paediatric condition.
Keywords: refractory epilepsy, Febrile illness-related epilepsy syndrome, anakinra, induced-hypothermia, Ketogenic Diet, New onset refractory status epilepticus, antiepileptic drugs
Received: 01 May 2025; Accepted: 07 Oct 2025.
Copyright: © 2025 Caporale, Lais, Gulli', Caporale, Piastra and Conti. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Mariagiovanna Caporale, mariagiovanna.caporale@guest.policlinicogemelli.it
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