How I treat pediatric febrile infection-related epilepsy syndrome

Xiao-Lu DENG, Jing PENG

Chinese Journal of Contemporary Pediatrics ›› 2026, Vol. 28 ›› Issue (6) : 659-664.

PDF(613 KB)
PDF(613 KB)
Chinese Journal of Contemporary Pediatrics ›› 2026, Vol. 28 ›› Issue (6) : 659-664. DOI: 10.7499/j.issn.1008-8830.2512090
HOW I TREAT

How I treat pediatric febrile infection-related epilepsy syndrome

Author information +
History +

Abstract

Febrile infection-related epilepsy syndrome (FIRES) mainly affects previously healthy children and adolescents, often leading to severe neurological impairment and long-term sequelae such as drug-resistant epilepsy and cognitive dysfunction. Based on two pediatric FIRES cases, combined with international and domestic guidelines as well as clinical experience, this paper highlights therapeutic strategies and escalation pathways tailored to different inflammatory phases in the acute and chronic stages. Incorporating continuous electroencephalogram monitoring and cerebrospinal fluid inflammatory cytokine profile changes, a phase-specific treatment approach is systematically described, centered on the rapid termination of status epilepticus, targeted cytokine immunotherapy, and multidisciplinary collaborative support, and the active use of tocilizumab, anakinra, and the ketogenic diet is emphasized, to provide practical guidance for clinicians in managing FIRES.

Key words

Febrile infection-related epilepsy syndrome / Status epilepticus / Diagnosis / Immunotherapy / Child

Cite this article

Download Citations
Xiao-Lu DENG , Jing PENG. How I treat pediatric febrile infection-related epilepsy syndrome[J]. Chinese Journal of Contemporary Pediatrics. 2026, 28(6): 659-664 https://doi.org/10.7499/j.issn.1008-8830.2512090

References

[1]
Wickstrom R, Taraschenko O, Dilena R, et al. International consensus recommendations for management of new onset refractory status epilepticus (NORSE) including febrile Infection-related epilepsy syndrome (FIRES): summary and clinical tools[J]. Epilepsia, 2022, 63(11): 2827-2839. PMCID: PMC9826478. DOI: 10.1111/epi.17391 .
[2]
Koh S, Wirrell E, Vezzani A, et al. Proposal to optimize evaluation and treatment of febrile infection-related epilepsy syndrome (FIRES): a report from FIRES workshop[J]. Epilepsia open, 2021, 6(1): 62-72. PMCID: PMC7918329. DOI: 10.1002/epi4.12447 .
[3]
van Baalen A. Febrile infection-related epilepsy syndrome in childhood: a clinical review and practical approach[J]. Seizure, 2023, 111: 215-222. DOI: 10.1016/j.seizure.2023.09.008 .
[4]
Gettings JV, Mohammad Alizadeh Chafjiri F, Patel AA, et al. Diagnosis and management of status epilepticus: improving the status quo[J]. Lancet Neurol, 2025, 24(1): 65-76. DOI: 10.1016/S1474-4422(24)00430-7 .
[5]
儿童癫痫持续状态协作组. 儿童癫痫持续状态诊断治疗的中国专家共识(2022)[J]. 癫痫杂志, 2022, 8(5): 383-389.
[6]
王萱, 江文. 新发难治性癫痫持续状态/热性感染相关性癫痫综合征: 癫痫持续状态诊疗的新挑战[J]. 中华神经科杂志, 2024, 57(4): 309-314. DOI: 10.3760/cma.j.cn113694-20231030-00280 .
[7]
Jun JS, Lee ST, Kim R, et al. Tocilizumab treatment for new onset refractory status epilepticus[J]. Ann Neurol, 2018, 84(6): 940-945. DOI: 10.1002/ana.25374 .
[8]
Giussani G, Bianchi E, Bonardi CM, et al. Retrospective multicenter study on cryptogenic NORSE/FIRES patients treated with anakinra[J]. Seizure, 2025, 130: 141-146. DOI: 10.1016/j.seizure.2025.06.022 .
[9]
Horino A, Kuki I, Inoue T, et al. Intrathecal dexamethasone therapy for febrile infection-related epilepsy syndrome[J]. Ann Clin Transl Neurol, 2021, 8(3): 645-655. PMCID: PMC7951105. DOI: 10.1002/acn3.51308 .
[10]
Hanin A, Muscal E, Hirsch LJ. Second-line immunotherapy in new onset refractory status epilepticus[J]. Epilepsia, 2024, 65(5): 1203-1223. DOI: 10.1111/epi.17933 .
[11]
Peng P, Peng J, Yin F, et al. Ketogenic diet as a treatment for super-refractory status epilepticus in febrile infection-related epilepsy syndrome[J]. Front Neurol, 2019, 10: 423. PMCID: PMC6498987. DOI: 10.3389/fneur.2019.00423 .
[12]
Norelli M, Camisa B, Barbiera G, et al. Monocyte-derived IL-1 and IL-6 are differentially required for cytokine-release syndrome and neurotoxicity due to CAR T cells[J]. Nat Med, 2018, 24(6): 739-748. DOI: 10.1038/s41591-018-0036-4 .
[13]
Aledo-Serrano A, Hariramani R, Gonzalez-Martinez A, et al. Anakinra and tocilizumab in the chronic phase of febrile infection-related epilepsy syndrome (FIRES): effectiveness and safety from a case-series[J]. Seizure, 2022, 100: 51-55. DOI: 10.1016/j.seizure.2022.06.012 .
[14]
Feng L, Li H, Ma L, et al. Minocycline in chronic management of febrile infection-related epilepsy syndrome (FIRES): a case series and literature review of treatment strategies[J]. Acta Epileptol, 2025, 7(1): 35. PMCID: PMC12142932. DOI: 10.1186/s42494-025-00224-4 .
[15]
Gofshteyn JS, Wilfong A, Devinsky O, et al. Cannabidiol as a potential treatment for febrile infection-related epilepsy syndrome (FIRES) in the acute and chronic phases[J]. J Child Neurol, 2017, 32(1): 35-40. DOI: 10.1177/0883073816669450 .
[16]
Surí-Báez C, Kim HJ, Lester J, et al. Beyond anakinra and tocilizumab: additional adjunctive therapies in pediatric new onset refractory status epilepticus and febrile infection-related epilepsy syndrome: a narrative review[J]. Pediatr Neurol, 2026, 175: 27-39. DOI: 10.1016/j.pediatrneurol.2025.10.022 .
[17]
Liu T, Li Z, Xu J, et al. Vagus nerve stimulation and fast parameter programming protocol in children with febrile infection-related epilepsy syndrome in ICU[J]. Neurotherapeutics, 2025, 22(3): e00551. PMCID: PMC12047395. DOI: 10.1016/j.neurot.2025.e00551 .

Footnotes

所有作者均声明无利益冲突。

PDF(613 KB)

Accesses

Citation

Detail

Sections
Recommended

/