我如何治疗儿童热性感染相关性癫痫综合征

邓小鹿, 彭镜

中国当代儿科杂志 ›› 2026, Vol. 28 ›› Issue (6) : 659-664.

PDF(613 KB)
HTML
PDF(613 KB)
HTML
中国当代儿科杂志 ›› 2026, Vol. 28 ›› Issue (6) : 659-664. DOI: 10.7499/j.issn.1008-8830.2512090
我的治疗策略

我如何治疗儿童热性感染相关性癫痫综合征

作者信息 +

How I treat pediatric febrile infection-related epilepsy syndrome

Author information +
文章历史 +

摘要

热性感染相关性癫痫综合征(febrile infection‑related epilepsy syndrome, FIRES)主要累及既往健康的儿童及青少年,常伴随严重的神经功能损伤,遗留药物难治性癫痫、认知障碍等远期后遗症。该文基于2例儿童FIRES病例,结合国际、国内指南和临床实践经验,重点展示急性期与慢性期不同炎症阶段下的治疗策略及其升级路径。结合持续脑电图监测及脑脊液炎症因子谱变化,系统阐述以快速终止癫痫持续状态-靶向细胞因子免疫治疗-多学科协同支持为核心的分阶段治疗思路,并重点强调托珠单抗、阿那白滞素及生酮饮食的积极应用,以期为临床医师在FIRES中的治疗决策提供可操作参考

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.

关键词

热性感染相关性癫痫综合征 / 癫痫持续状态 / 诊断 / 免疫治疗 / 儿童

Key words

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

引用本文

导出引用
邓小鹿, 彭镜. 我如何治疗儿童热性感染相关性癫痫综合征[J]. 中国当代儿科杂志. 2026, 28(6): 659-664 https://doi.org/10.7499/j.issn.1008-8830.2512090
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

参考文献

[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 .

脚注

所有作者均声明无利益冲突。

基金

国家自然科学基金(82471488)
湖南省自然科学基金(2026JJ80167)
中南大学教改课题(2025jy203)

版权

版权所有 © 2023中国当代儿科杂志
PDF(613 KB)
HTML

Accesses

Citation

Detail

段落导航
相关文章

/