Research progress on acute phase care of febrile infection-related epilepsy syndrome in children_Journal of Epilepsy

Authors：

WANG Fangping ,  TIAN Xiaoqin , GUO Xiaocui , YE Jinghua , ZHANG Jingxuan

Department of Neurology, Shenzhen Children’s Hospital, Shenzhen 518038, China;

Corresponding author：

TIAN Xiaoqin, Email: 110985685@qq.com

Keywords：

Febrile infection-related epilepsy syndrome; Status epilepticus; Ketogenic diet; Nursing; Review

DOI：

10.7507/2096-0247.202502009

Video：

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Abstract Full text Figures/Tables Video References Cited by

Febrile infection-related epilepsy syndrome (FIRES) is a rare and severe epileptic encephalopathy characterized by critical illness, complex nursing requirements, the need for multidisciplinary collaboration, and high-intensity care during its acute phase. Based on a review of relevant literature and specific nursing practices, this article summarizes the latest advancements in the acute-phase care of children with FIRES. It focuses on aspects such as the management of status epilepticus, fever care, airway management, nutritional support and ketogenic diet, family support, and multidisciplinary collaboration. The aim is to provide a reference for clinical nursing practices and related research.

Citation： WANG Fangping, TIAN Xiaoqin, GUO Xiaocui, YE Jinghua, ZHANG Jingxuan. Research progress on acute phase care of febrile infection-related epilepsy syndrome in children. Journal of Epilepsy, 2025, 11(3): 261-265. doi: 10.7507/2096-0247.202502009 Copy

1.	牟常华, 周昀箐, 王纪文. 热性感染相关性癫痫综合征的诊治研究进展. 癫痫杂志, 2017, 3(6): 519-521.
2.	Kramer U, Shorer Z, Ben-Zeev B, et al. Severe refractory status epilepticus owing to presumed encephalitis. J Child Neurol, 2005, 20(3): 184-187.
3.	Baxter P, Clarke A, Cross H, et al. Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status. Seizure, 2003, 12(6): 379-387.
4.	van Baalen A, Häusler M, Boor R, et al. Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood. Epilepsia, 2010, 51(7): 1323-1328.
5.	Gaspard N, Hirsch L J, Sculier C, et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): state of the art and perspectives. Epilepsia, 2018, 59(4): 745-752.
6.	Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia, 2018, 59(4): 739-744.
7.	Howell KB, Katanyuwong K, Mackay MT, et al. Long-term follow-up of febrile infection-related epilepsy syndrome. Epilepsia, 2012, 53(1): 101-110.
8.	Vezzani A, Balosso S, Ravizza T. Neuroinflammatory pathways as treatment targets and biomarkers in epilepsy. Nat Rev Neurol, 2019, 1 5(8): 459-472.
9.	Hon KL, Leung A, Torres AR. Febrile infection-related epilepsy syndrome (fires): an overview of treatment and recent patents. Recent Pat Inflamm Allergy Drug Discov, 2018, 12(2): 128-135.
10.	Wickstrom R, Taraschenko O, Dilena R, et al. International consensus recommendations for management of new onset refractory status epilepticus including febrileinfection‐related epilepsy syndrome: statements and supporting evidence. Epilepsia, 2022, 63(11): 2840-2864.
11.	Specchio N, Pietrafusa N. New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. Dev Med Child Neurol, 2020, 62(8): 897-905.
12.	van Baalen A. Febrile infection-related epilepsy syndrome in childhood: a clinical review and practical approach. Seizure, 2023, 111: 215-222.
13.	屈素清, 刘旭斌, 李静, 等. 热性感染相关性癫痫综合征亚急性期的序贯治疗及转归. 卒中与神经疾病, 2022, 29(2): 168-171.
14.	胡红玲, 周霞. 发热感染相关性癫痫综合征的临床护理. 齐鲁护理杂志, 2020, 26(5): 110-112.
15.	王海勤, 王丽娟, 李红翠, 等. 热性感染相关性癫痫综合征患儿的护理. 护士进修杂志, 2019, 34(8): 721-723.
16.	Sheikh Z, Hirsch LJ. A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome. Front Neurol, 2023, 14: 1150496.
17.	Pinto LF, Oliveira JPS, Midon AM. Status epilepticus: review on diagnosis, monitoring and treatment. Arq Neuropsiquiatr, 2022, 80(Suppl 1): 193-203.
18.	刘秀芬, 徐桂霞, 魏素珍. 集束化护理在持续静脉泵入咪达唑仑治疗儿童癫痫持续状态中的应用. 齐鲁护理杂志, 2017, 23(23): 90-92.
19.	赵璠, 杨芳, 贺琳晰. 复方黄柏液在PICU患儿持续静点咪达唑仑所致静脉炎中的应用分析. 实用药物与临床, 2019, 22(8): 846-849.
20.	吴晔, 桑田, 管巧. 热性感染相关性癫痫综合征. 重庆医科大学学报, 2025, 1-5. Epub ahead of print.
21.	Diringer MN, Reaven NL, Funk SE, et al. Elevated body temperature independently contributes to increased length of stay in neurologic intensive care unit patients. Crit Care Med, 2004, 32(7): 1489-1495.
22.	李之晗. 发热控制对神经重症患者脑血流自动调节能力的影响研究. 中南大学, 硕士学位论文, 2022.
23.	Posthuma LM, Breteler M, Lirk PB, et al. Surveillance of high-risk early postsurgical patients for real-time detection of complications using wireless monitoring (SHEPHERD study): results of a randomized multicenter stepped wedge cluster trial. Front Med (Lausanne), 2023, 10: 1295499.
24.	国家呼吸系统疾病临床医学研究中心, 中华医学会儿科学分会呼吸学组, 中国医师协会呼吸医师分会儿科呼吸工作委员会, 等. 解热镇痛药在儿童发热对症治疗中的合理用药专家共识. 中华实用儿科临床杂志, 2020, 35(3): 161-169.
25.	中国医师协会神经外科分会神经重症专家委员会, 北京医学会神经外科分会神经外科危重症学组, 中国神经外科重症管理协作组. 神经重症目标温度管理中国专家共识(2022版). 中华神经医学杂志, 2022, 21(7): 649-656.
26.	中国研究型医院学会神经再生与修复专业委员会心脏重症脑保护学组, 中国研究型医院学会神经再生与修复专业委员会神经重症护理与康复学组. 亚低温脑保护中国专家共识. 中华危重病急救医学, 2020, 32(4): 385-391.
27.	Lin JJ, Lin KL, Hsia SH, et al. Therapeutic hypothermia for febrile infection-related epilepsy syndrome in two patients. Pediatr Neurol, 2012, 47(6): 448-450.
28.	Legriel S. Hypothermia as a treatment in status epilepticus: a narrative review. Epilepsy Behav, 2019, 101(Pt B): 106298.
29.	吕云霞, 冯少娟, 刘正伟, 等. 气管切开术后非机械通气患儿早期气道并发症预防的最佳证据总结. 护理学报, 2021, 28(13): 46-51.
30.	赵海平, 王荣荣. 神经重症气管切开患者气道功能康复与管理专家共识(2024). 中国康复理论与实践, 2024, 30(8): 869-881.
31.	邱茂青, 黄淑芬. 早期肠内营养治疗对儿童重症监护室患儿临床结局的影响. 妇儿健康导刊, 2024, 3(19): 37-41.
32.	李猛, 朱刚, 梁桂珍. 不同肠内营养对儿童重症病毒性脑炎治疗过程中的辅助作用. 热带医学杂志, 2013, 13(6): 765-767.
33.	李晓玉, 曾慧琳, 李慧强, 等. 儿童经皮内镜胃造瘘术造口皮肤并发症管理的最佳证据总结. 中华现代护理杂志, 2024, 30(22): 2978-2983.
34.	耿岚岚, 谢静. 儿童肠内营养应用的研究进展. 中华实用儿科临床杂志, 2019, 34(7): 481-484.
35.	邓瑶, 喻韬, 陈小璐, 等. 生酮饮食治疗热性感染相关性癫痫综合征5例并文献复习. 癫痫杂志, 2021, 7(4): 281-287.
36.	廖建湘. 生酮饮食疗法在癫痫及相关神经系统疾病中的应用专家共识. 中华儿科杂志, 2019, 57(11): 820-825.
37.	马变慧, 廖建湘. 生酮饮食疗法的不良反应及防治的研究进展. 国际儿科学杂志, 2023, 50(4): 272-276.
38.	Bandi R, Jain V, Lingappa L, et al. Challenges in management of febrile infection-related epilepsy syndrome: real-world experience from a large cohort of pediatric patients. Pediatr Neurol, 2025, 165: 9-15.
39.	Yoneno S, Baba S, Sumitomo N, et al. The efficacy of a ketogenic diet in a case with febrile infection-related epilepsy syndrome in the chronic phase: a case report. Cureus, 2024, 16(7): e64149.
40.	中国抗癫痫协会护理专业委员会. 癫痫健康教育中国护理专家共识. 癫痫杂志, 2024, 10(6): 495-502.
41.	郭松领, 颜莉, 郑显兰, 等. 儿童癫痫急性发作院外管理的最佳证据总结. 护士进修杂志, 2024, 39(16): 1754-1759.
42.	张捷, 刘伟, 邓泂, 等. 儿童热性感染相关性癫痫综合征的临床特征和预后分析. 重庆医科大学学报, 2025, 1-7. Epub ahead of print.
43.	孙琳琳, 叶敬花, 王芳萍. 儿童医疗辅导游戏对提高难治性癫痫患儿生酮饮食治疗依从性和酮症状态的效果研究. 癫痫杂志, 2024, 10(6): 508-513.
44.	白福秀, 李素丽, 李变霞. 大剂量苯巴比妥救治热性感染相关性癫痫综合征的护理体会. 中国药物与临床, 2016, 16(7): 1088-1089.

1. 牟常华, 周昀箐, 王纪文. 热性感染相关性癫痫综合征的诊治研究进展. 癫痫杂志, 2017, 3(6): 519-521.
2. Kramer U, Shorer Z, Ben-Zeev B, et al. Severe refractory status epilepticus owing to presumed encephalitis. J Child Neurol, 2005, 20(3): 184-187.
3. Baxter P, Clarke A, Cross H, et al. Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status. Seizure, 2003, 12(6): 379-387.
4. van Baalen A, Häusler M, Boor R, et al. Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood. Epilepsia, 2010, 51(7): 1323-1328.
5. Gaspard N, Hirsch L J, Sculier C, et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): state of the art and perspectives. Epilepsia, 2018, 59(4): 745-752.
6. Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia, 2018, 59(4): 739-744.
7. Howell KB, Katanyuwong K, Mackay MT, et al. Long-term follow-up of febrile infection-related epilepsy syndrome. Epilepsia, 2012, 53(1): 101-110.
8. Vezzani A, Balosso S, Ravizza T. Neuroinflammatory pathways as treatment targets and biomarkers in epilepsy. Nat Rev Neurol, 2019, 1 5(8): 459-472.
9. Hon KL, Leung A, Torres AR. Febrile infection-related epilepsy syndrome (fires): an overview of treatment and recent patents. Recent Pat Inflamm Allergy Drug Discov, 2018, 12(2): 128-135.
10. Wickstrom R, Taraschenko O, Dilena R, et al. International consensus recommendations for management of new onset refractory status epilepticus including febrileinfection‐related epilepsy syndrome: statements and supporting evidence. Epilepsia, 2022, 63(11): 2840-2864.
11. Specchio N, Pietrafusa N. New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome. Dev Med Child Neurol, 2020, 62(8): 897-905.
12. van Baalen A. Febrile infection-related epilepsy syndrome in childhood: a clinical review and practical approach. Seizure, 2023, 111: 215-222.
13. 屈素清, 刘旭斌, 李静, 等. 热性感染相关性癫痫综合征亚急性期的序贯治疗及转归. 卒中与神经疾病, 2022, 29(2): 168-171.
14. 胡红玲, 周霞. 发热感染相关性癫痫综合征的临床护理. 齐鲁护理杂志, 2020, 26(5): 110-112.
15. 王海勤, 王丽娟, 李红翠, 等. 热性感染相关性癫痫综合征患儿的护理. 护士进修杂志, 2019, 34(8): 721-723.
16. Sheikh Z, Hirsch LJ. A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome. Front Neurol, 2023, 14: 1150496.
17. Pinto LF, Oliveira JPS, Midon AM. Status epilepticus: review on diagnosis, monitoring and treatment. Arq Neuropsiquiatr, 2022, 80(Suppl 1): 193-203.
18. 刘秀芬, 徐桂霞, 魏素珍. 集束化护理在持续静脉泵入咪达唑仑治疗儿童癫痫持续状态中的应用. 齐鲁护理杂志, 2017, 23(23): 90-92.
19. 赵璠, 杨芳, 贺琳晰. 复方黄柏液在PICU患儿持续静点咪达唑仑所致静脉炎中的应用分析. 实用药物与临床, 2019, 22(8): 846-849.
20. 吴晔, 桑田, 管巧. 热性感染相关性癫痫综合征. 重庆医科大学学报, 2025, 1-5. Epub ahead of print.
21. Diringer MN, Reaven NL, Funk SE, et al. Elevated body temperature independently contributes to increased length of stay in neurologic intensive care unit patients. Crit Care Med, 2004, 32(7): 1489-1495.
22. 李之晗. 发热控制对神经重症患者脑血流自动调节能力的影响研究. 中南大学, 硕士学位论文, 2022.
23. Posthuma LM, Breteler M, Lirk PB, et al. Surveillance of high-risk early postsurgical patients for real-time detection of complications using wireless monitoring (SHEPHERD study): results of a randomized multicenter stepped wedge cluster trial. Front Med (Lausanne), 2023, 10: 1295499.
24. 国家呼吸系统疾病临床医学研究中心, 中华医学会儿科学分会呼吸学组, 中国医师协会呼吸医师分会儿科呼吸工作委员会, 等. 解热镇痛药在儿童发热对症治疗中的合理用药专家共识. 中华实用儿科临床杂志, 2020, 35(3): 161-169.
25. 中国医师协会神经外科分会神经重症专家委员会, 北京医学会神经外科分会神经外科危重症学组, 中国神经外科重症管理协作组. 神经重症目标温度管理中国专家共识(2022版). 中华神经医学杂志, 2022, 21(7): 649-656.
26. 中国研究型医院学会神经再生与修复专业委员会心脏重症脑保护学组, 中国研究型医院学会神经再生与修复专业委员会神经重症护理与康复学组. 亚低温脑保护中国专家共识. 中华危重病急救医学, 2020, 32(4): 385-391.
27. Lin JJ, Lin KL, Hsia SH, et al. Therapeutic hypothermia for febrile infection-related epilepsy syndrome in two patients. Pediatr Neurol, 2012, 47(6): 448-450.
28. Legriel S. Hypothermia as a treatment in status epilepticus: a narrative review. Epilepsy Behav, 2019, 101(Pt B): 106298.
29. 吕云霞, 冯少娟, 刘正伟, 等. 气管切开术后非机械通气患儿早期气道并发症预防的最佳证据总结. 护理学报, 2021, 28(13): 46-51.
30. 赵海平, 王荣荣. 神经重症气管切开患者气道功能康复与管理专家共识(2024). 中国康复理论与实践, 2024, 30(8): 869-881.
31. 邱茂青, 黄淑芬. 早期肠内营养治疗对儿童重症监护室患儿临床结局的影响. 妇儿健康导刊, 2024, 3(19): 37-41.
32. 李猛, 朱刚, 梁桂珍. 不同肠内营养对儿童重症病毒性脑炎治疗过程中的辅助作用. 热带医学杂志, 2013, 13(6): 765-767.
33. 李晓玉, 曾慧琳, 李慧强, 等. 儿童经皮内镜胃造瘘术造口皮肤并发症管理的最佳证据总结. 中华现代护理杂志, 2024, 30(22): 2978-2983.
34. 耿岚岚, 谢静. 儿童肠内营养应用的研究进展. 中华实用儿科临床杂志, 2019, 34(7): 481-484.
35. 邓瑶, 喻韬, 陈小璐, 等. 生酮饮食治疗热性感染相关性癫痫综合征5例并文献复习. 癫痫杂志, 2021, 7(4): 281-287.
36. 廖建湘. 生酮饮食疗法在癫痫及相关神经系统疾病中的应用专家共识. 中华儿科杂志, 2019, 57(11): 820-825.
37. 马变慧, 廖建湘. 生酮饮食疗法的不良反应及防治的研究进展. 国际儿科学杂志, 2023, 50(4): 272-276.
38. Bandi R, Jain V, Lingappa L, et al. Challenges in management of febrile infection-related epilepsy syndrome: real-world experience from a large cohort of pediatric patients. Pediatr Neurol, 2025, 165: 9-15.
39. Yoneno S, Baba S, Sumitomo N, et al. The efficacy of a ketogenic diet in a case with febrile infection-related epilepsy syndrome in the chronic phase: a case report. Cureus, 2024, 16(7): e64149.
40. 中国抗癫痫协会护理专业委员会. 癫痫健康教育中国护理专家共识. 癫痫杂志, 2024, 10(6): 495-502.
41. 郭松领, 颜莉, 郑显兰, 等. 儿童癫痫急性发作院外管理的最佳证据总结. 护士进修杂志, 2024, 39(16): 1754-1759.
42. 张捷, 刘伟, 邓泂, 等. 儿童热性感染相关性癫痫综合征的临床特征和预后分析. 重庆医科大学学报, 2025, 1-7. Epub ahead of print.
43. 孙琳琳, 叶敬花, 王芳萍. 儿童医疗辅导游戏对提高难治性癫痫患儿生酮饮食治疗依从性和酮症状态的效果研究. 癫痫杂志, 2024, 10(6): 508-513.
44. 白福秀, 李素丽, 李变霞. 大剂量苯巴比妥救治热性感染相关性癫痫综合征的护理体会. 中国药物与临床, 2016, 16(7): 1088-1089.

Journal of Epilepsy

Research progress on acute phase care of febrile infection-related epilepsy syndrome in children

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