热性感染相关性癫痫综合征的诊治研究进展_Journal of Epilepsy

Authors：

牟常华 , 周昀箐 ,  王纪文

上海交通大学医学院附属上海儿童医学中心国家儿童医学中心神经内科(上海200127);

Corresponding author：

王纪文, Email: wangjiwen_99@hotmail.com

Keywords：

发热; 癫痫持续状态; 癫痫性脑病; 生酮饮食

DOI：

10.7507/2096-0247.20170084

Video：

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热性感染相关性癫痫综合征(Febrile infection-related epilepsy syndrome，FIRES)是近年来逐渐被人们认识的最严重癫痫性脑病之一，主要特征是急性热性疾病后数天出现难治性癫痫持续状态或成簇癫痫发作，并随即演变为局灶性发作为主的药物难治性癫痫和认知损害等神经心理学障碍。FIRES的病因和发病机制尚不完全清楚，可能是一种免疫性但不是自身免疫性疾病。其诊断主要依据其临床特征和排除其他相关性疾病，治疗十分困难，预后不良。但FIRES的早期诊断至关重要，可以指导治疗，尤其是选择最佳的一线治疗，有助于改善预后。

Citation： 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. Journal of Epilepsy, 2017, 3(6): 519-521. doi: 10.7507/2096-0247.20170084 Copy

1.	Lyon G, Dodge PR, Adams RD. The acute encephalopathies of obscure origin in infants and children. Brain, 1961, 84: 680-708.
2.	Sakuma H, Fukumizu M, Kohyama J. Efficacy of anticonvulsants on acute encephalitis with refractory, repetitive partial seizures (AERRPS). No ToHattatsu, 2001, 33(5): 385-390.
3.	Mikaeloff Y, Jambaqué I, Hertz-Pannier L, et al. Devastating epileptic encephalopathy in school-aged children (DESC): A pseudo encephalitis. Epilepsy Res, 2006, 69(1): 67-79.
4.	van Baalen A, Vezzani A, H usler M, et al. Febrile Infection-Related Epilepsy Syndrome: Clinical Review and Hypotheses of Epileptogenesis. Neuropediatrics, 2017, 48(1): 5-18.
5.	Ismail FY, Kossoff EH. AERRPS, DESC, NORSE, FIRES: multi-labeling or distinct epileptic entities.Epilepsia, 2011, 52(11): e185-e189.
6.	Sato Y, Numata-Uematsu Y, Uematsu M, et al. Acute encephalitis with refractory, repetitive partial seizures: pathological findings and A new therapeutic approach using tacrolimus. Brain Dev, 2016, 38(8): 772-776.
7.	van Baalen A, H usler M, Plecko-Startinig B, et al. Febrile infection-related epilepsy syndrome without detectable autoantibodies and response to immunotherapy: A case series and discussion of epileptogenesis in FIRES. Neuropediatrics, 2012, 43(4): 209-216.
8.	Kramer U, Chi CS, Lin KL, et al. Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: A multicenter study on 77 children. Epilepsia, 2011, 52(11): 1956-1965.
9.	Sutter R, Kaplan PW. Can anesthetic treatment worsen outcome in status epilepticus. Epilepsy Behav, 2015, 49: 294-297.
10.	Bellante F, Legros B, Depondt C, et al. Midazolam and thiopental for the treatment of refractory status epilepticus: A retrospective comparison of efficacy and safety. J Neurol, 2016, 263(4): 799-806.
11.	Nabbout R, Mazzuca M, Hubert P, et al. Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES). Epilepsia, 2010, 51(10): 2033-2037.
12.	Singh RK, Joshi SM, Potter DM, et al. Cognitive outcomes in febrile infection-related epilepsy syndrome treated with the ketogenic diet. Pediatrics, 2014, 134(5): e1431-e1435.
13.	Chang P, Augustin K, Boddum K, et al. Seizure control by decanoic acid through direct AMPA receptor inhibition. Brain, 2016, 139(Pt 2): 431-443.
14.	Dupuis N, Curatolo N, Benoist JF, et al. Ketogenic diet exhibits anti-inflammatory properties. Epilepsia, 2015, 56(7): e95-e98.
15.	Farias-Moeller R, Bartolini L, Pasupuleti A, et al. A practical approach to ketogenic diet in the pediatric intensive care unit for super-refractory status epilepticus. Neurocrit Care, 2017, 26(2): 267-272.
16.	Baumeister FA, Oberhoffer R, Liebhaber GM, et al. Fatal propofol infusion syndrome in association with ketogenic diet. Neuropediatrics, 2004, 35(4): 250-252.
17.	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 Child Neurol, 2017, 32(1): 35-40.

1. Lyon G, Dodge PR, Adams RD. The acute encephalopathies of obscure origin in infants and children. Brain, 1961, 84: 680-708.
2. Sakuma H, Fukumizu M, Kohyama J. Efficacy of anticonvulsants on acute encephalitis with refractory, repetitive partial seizures (AERRPS). No ToHattatsu, 2001, 33(5): 385-390.
3. Mikaeloff Y, Jambaqué I, Hertz-Pannier L, et al. Devastating epileptic encephalopathy in school-aged children (DESC): A pseudo encephalitis. Epilepsy Res, 2006, 69(1): 67-79.
4. van Baalen A, Vezzani A, H usler M, et al. Febrile Infection-Related Epilepsy Syndrome: Clinical Review and Hypotheses of Epileptogenesis. Neuropediatrics, 2017, 48(1): 5-18.
5. Ismail FY, Kossoff EH. AERRPS, DESC, NORSE, FIRES: multi-labeling or distinct epileptic entities.Epilepsia, 2011, 52(11): e185-e189.
6. Sato Y, Numata-Uematsu Y, Uematsu M, et al. Acute encephalitis with refractory, repetitive partial seizures: pathological findings and A new therapeutic approach using tacrolimus. Brain Dev, 2016, 38(8): 772-776.
7. van Baalen A, H usler M, Plecko-Startinig B, et al. Febrile infection-related epilepsy syndrome without detectable autoantibodies and response to immunotherapy: A case series and discussion of epileptogenesis in FIRES. Neuropediatrics, 2012, 43(4): 209-216.
8. Kramer U, Chi CS, Lin KL, et al. Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: A multicenter study on 77 children. Epilepsia, 2011, 52(11): 1956-1965.
9. Sutter R, Kaplan PW. Can anesthetic treatment worsen outcome in status epilepticus. Epilepsy Behav, 2015, 49: 294-297.
10. Bellante F, Legros B, Depondt C, et al. Midazolam and thiopental for the treatment of refractory status epilepticus: A retrospective comparison of efficacy and safety. J Neurol, 2016, 263(4): 799-806.
11. Nabbout R, Mazzuca M, Hubert P, et al. Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES). Epilepsia, 2010, 51(10): 2033-2037.
12. Singh RK, Joshi SM, Potter DM, et al. Cognitive outcomes in febrile infection-related epilepsy syndrome treated with the ketogenic diet. Pediatrics, 2014, 134(5): e1431-e1435.
13. Chang P, Augustin K, Boddum K, et al. Seizure control by decanoic acid through direct AMPA receptor inhibition. Brain, 2016, 139(Pt 2): 431-443.
14. Dupuis N, Curatolo N, Benoist JF, et al. Ketogenic diet exhibits anti-inflammatory properties. Epilepsia, 2015, 56(7): e95-e98.
15. Farias-Moeller R, Bartolini L, Pasupuleti A, et al. A practical approach to ketogenic diet in the pediatric intensive care unit for super-refractory status epilepticus. Neurocrit Care, 2017, 26(2): 267-272.
16. Baumeister FA, Oberhoffer R, Liebhaber GM, et al. Fatal propofol infusion syndrome in association with ketogenic diet. Neuropediatrics, 2004, 35(4): 250-252.
17. 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 Child Neurol, 2017, 32(1): 35-40.

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