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：

Export PDF Favorites Scan Get Citation

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.	牟常華, 周昀箐, 王紀文. 熱性感染相關性癲癇綜合征的診治研究進展. 癲癇雜志, 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, H?usler 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, 劉旭斌, 李靜, 等. 熱性感染相關性癲癇綜合征亞急性期的序貫治療及轉歸. 卒中與神經疾病, 2022, 29(2): 168-171.
14.	胡紅玲, 周霞. 發熱感染相關性癲癇綜合征的臨床護理. 齊魯護理雜志, 2020, 26(5): 110-112.
15.	王海勤, 王麗娟, 李紅翠, 等. 熱性感染相關性癲癇綜合征患兒的護理. 護士進修雜志, 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.	劉秀芬, 徐桂霞, 魏素珍. 集束化護理在持續靜脈泵入咪達唑侖治療兒童癲癇持續狀態中的應用. 齊魯護理雜志, 2017, 23(23): 90-92.
19.	趙璠, 楊芳, 賀琳晰. 復方黃柏液在PICU患兒持續靜點咪達唑侖所致靜脈炎中的應用分析. 實用藥物與臨床, 2019, 22(8): 846-849.
20.	吳曄, 桑田, 管巧. 熱性感染相關性癲癇綜合征. 重慶醫科大學學報, 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. 發熱控制對神經重癥患者腦血流自動調節能力的影響研究. 中南大學, 碩士學位論文, 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.	國家呼吸系統疾病臨床醫學研究中心, 中華醫學會兒科學分會呼吸學組, 中國醫師協會呼吸醫師分會兒科呼吸工作委員會, 等. 解熱鎮痛藥在兒童發熱對癥治療中的合理用藥專家共識. 中華實用兒科臨床雜志, 2020, 35(3): 161-169.
25.	中國醫師協會神經外科分會神經重癥專家委員會, 北京醫學會神經外科分會神經外科危重癥學組, 中國神經外科重癥管理協作組. 神經重癥目標溫度管理中國專家共識(2022版). 中華神經醫學雜志, 2022, 21(7): 649-656.
26.	中國研究型醫院學會神經再生與修復專業委員會心臟重癥腦保護學組, 中國研究型醫院學會神經再生與修復專業委員會神經重癥護理與康復學組. 亞低溫腦保護中國專家共識. 中華危重病急救醫學, 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.	呂云霞, 馮少娟, 劉正偉, 等. 氣管切開術后非機械通氣患兒早期氣道并發癥預防的最佳證據總結. 護理學報, 2021, 28(13): 46-51.
30.	趙海平, 王榮榮. 神經重癥氣管切開患者氣道功能康復與管理專家共識(2024). 中國康復理論與實踐, 2024, 30(8): 869-881.
31.	邱茂青, 黃淑芬. 早期腸內營養治療對兒童重癥監護室患兒臨床結局的影響. 婦兒健康導刊, 2024, 3(19): 37-41.
32.	李猛, 朱剛, 梁桂珍. 不同腸內營養對兒童重癥病毒性腦炎治療過程中的輔助作用. 熱帶醫學雜志, 2013, 13(6): 765-767.
33.	李曉玉, 曾慧琳, 李慧強, 等. 兒童經皮內鏡胃造瘺術造口皮膚并發癥管理的最佳證據總結. 中華現代護理雜志, 2024, 30(22): 2978-2983.
34.	耿嵐嵐, 謝靜. 兒童腸內營養應用的研究進展. 中華實用兒科臨床雜志, 2019, 34(7): 481-484.
35.	鄧瑤, 喻韜, 陳小璐, 等. 生酮飲食治療熱性感染相關性癲癇綜合征5例并文獻復習. 癲癇雜志, 2021, 7(4): 281-287.
36.	廖建湘. 生酮飲食療法在癲癇及相關神經系統疾病中的應用專家共識. 中華兒科雜志, 2019, 57(11): 820-825.
37.	馬變慧, 廖建湘. 生酮飲食療法的不良反應及防治的研究進展. 國際兒科學雜志, 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.	中國抗癲癇協會護理專業委員會. 癲癇健康教育中國護理專家共識. 癲癇雜志, 2024, 10(6): 495-502.
41.	郭松領, 顏莉, 鄭顯蘭, 等. 兒童癲癇急性發作院外管理的最佳證據總結. 護士進修雜志, 2024, 39(16): 1754-1759.
42.	張捷, 劉偉, 鄧泂, 等. 兒童熱性感染相關性癲癇綜合征的臨床特征和預后分析. 重慶醫科大學學報, 2025, 1-7. Epub ahead of print.
43.	孫琳琳, 葉敬花, 王芳萍. 兒童醫療輔導游戲對提高難治性癲癇患兒生酮飲食治療依從性和酮癥狀態的效果研究. 癲癇雜志, 2024, 10(6): 508-513.
44.	白福秀, 李素麗, 李變霞. 大劑量苯巴比妥救治熱性感染相關性癲癇綜合征的護理體會. 中國藥物與臨床, 2016, 16(7): 1088-1089.

1. 牟常華, 周昀箐, 王紀文. 熱性感染相關性癲癇綜合征的診治研究進展. 癲癇雜志, 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, H?usler 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, 劉旭斌, 李靜, 等. 熱性感染相關性癲癇綜合征亞急性期的序貫治療及轉歸. 卒中與神經疾病, 2022, 29(2): 168-171.
14. 胡紅玲, 周霞. 發熱感染相關性癲癇綜合征的臨床護理. 齊魯護理雜志, 2020, 26(5): 110-112.
15. 王海勤, 王麗娟, 李紅翠, 等. 熱性感染相關性癲癇綜合征患兒的護理. 護士進修雜志, 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. 劉秀芬, 徐桂霞, 魏素珍. 集束化護理在持續靜脈泵入咪達唑侖治療兒童癲癇持續狀態中的應用. 齊魯護理雜志, 2017, 23(23): 90-92.
19. 趙璠, 楊芳, 賀琳晰. 復方黃柏液在PICU患兒持續靜點咪達唑侖所致靜脈炎中的應用分析. 實用藥物與臨床, 2019, 22(8): 846-849.
20. 吳曄, 桑田, 管巧. 熱性感染相關性癲癇綜合征. 重慶醫科大學學報, 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. 發熱控制對神經重癥患者腦血流自動調節能力的影響研究. 中南大學, 碩士學位論文, 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. 國家呼吸系統疾病臨床醫學研究中心, 中華醫學會兒科學分會呼吸學組, 中國醫師協會呼吸醫師分會兒科呼吸工作委員會, 等. 解熱鎮痛藥在兒童發熱對癥治療中的合理用藥專家共識. 中華實用兒科臨床雜志, 2020, 35(3): 161-169.
25. 中國醫師協會神經外科分會神經重癥專家委員會, 北京醫學會神經外科分會神經外科危重癥學組, 中國神經外科重癥管理協作組. 神經重癥目標溫度管理中國專家共識(2022版). 中華神經醫學雜志, 2022, 21(7): 649-656.
26. 中國研究型醫院學會神經再生與修復專業委員會心臟重癥腦保護學組, 中國研究型醫院學會神經再生與修復專業委員會神經重癥護理與康復學組. 亞低溫腦保護中國專家共識. 中華危重病急救醫學, 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. 呂云霞, 馮少娟, 劉正偉, 等. 氣管切開術后非機械通氣患兒早期氣道并發癥預防的最佳證據總結. 護理學報, 2021, 28(13): 46-51.
30. 趙海平, 王榮榮. 神經重癥氣管切開患者氣道功能康復與管理專家共識(2024). 中國康復理論與實踐, 2024, 30(8): 869-881.
31. 邱茂青, 黃淑芬. 早期腸內營養治療對兒童重癥監護室患兒臨床結局的影響. 婦兒健康導刊, 2024, 3(19): 37-41.
32. 李猛, 朱剛, 梁桂珍. 不同腸內營養對兒童重癥病毒性腦炎治療過程中的輔助作用. 熱帶醫學雜志, 2013, 13(6): 765-767.
33. 李曉玉, 曾慧琳, 李慧強, 等. 兒童經皮內鏡胃造瘺術造口皮膚并發癥管理的最佳證據總結. 中華現代護理雜志, 2024, 30(22): 2978-2983.
34. 耿嵐嵐, 謝靜. 兒童腸內營養應用的研究進展. 中華實用兒科臨床雜志, 2019, 34(7): 481-484.
35. 鄧瑤, 喻韜, 陳小璐, 等. 生酮飲食治療熱性感染相關性癲癇綜合征5例并文獻復習. 癲癇雜志, 2021, 7(4): 281-287.
36. 廖建湘. 生酮飲食療法在癲癇及相關神經系統疾病中的應用專家共識. 中華兒科雜志, 2019, 57(11): 820-825.
37. 馬變慧, 廖建湘. 生酮飲食療法的不良反應及防治的研究進展. 國際兒科學雜志, 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. 中國抗癲癇協會護理專業委員會. 癲癇健康教育中國護理專家共識. 癲癇雜志, 2024, 10(6): 495-502.
41. 郭松領, 顏莉, 鄭顯蘭, 等. 兒童癲癇急性發作院外管理的最佳證據總結. 護士進修雜志, 2024, 39(16): 1754-1759.
42. 張捷, 劉偉, 鄧泂, 等. 兒童熱性感染相關性癲癇綜合征的臨床特征和預后分析. 重慶醫科大學學報, 2025, 1-7. Epub ahead of print.
43. 孫琳琳, 葉敬花, 王芳萍. 兒童醫療輔導游戲對提高難治性癲癇患兒生酮飲食治療依從性和酮癥狀態的效果研究. 癲癇雜志, 2024, 10(6): 508-513.
44. 白福秀, 李素麗, 李變霞. 大劑量苯巴比妥救治熱性感染相關性癲癇綜合征的護理體會. 中國藥物與臨床, 2016, 16(7): 1088-1089.

Journal of Epilepsy

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

Abstract Full text Figures/Tables Video References Cited by

Previous Article

Next Article

Format

Content