Febrile infection-related epilepsy syndrome: A study of 12 patients
Febrile infection-related epilepsy syndrome: A study of 12 patients
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DOI：
10.1016/j.seizure.2013.04.005
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发表时间：
2013-09-01
影响因子：
3
通讯作者：
Cersosimo, Ricardo
中科院分区：
文献类型：
--
作者：
Caraballo, Roberto H.;Reyes, Gabriela;Cersosimo, Ricardo
目的：分析12例发热性感染相关性癫痫综合征（FIRES）患者的电临床特征、神经影像学表现、治疗及转归。方法：回顾性研究了布宜诺斯艾利斯Garrahan医院1997年至2012年期间12例平均随访时间为6.5年的FIRES患儿。结果：8男4女局灶性癫痫持续状态伴发热性感染，平均发病年龄8.5岁。急性期全部采用抗癫痫药物治疗，10例采用免疫治疗，8例采用突发抑制性昏迷。生酮饮食在两个，血浆置换在一个，利妥昔单抗在一个。两名接受IVIG治疗的患者和一名接受类固醇治疗的患者有良好的反应，但在这个阶段，只有三名患者对IVIG和生酮饮食有长期的良好反应。在此期间没有患者死亡。在慢性癫痫期，所有儿童的癫痫发作都是由新皮质区引起的。所有患者均有难治性癫痫，多数为智力迟钝和行为障碍。所有患者都接受了不同的aed治疗，在这个阶段，第三名患者接受生酮饮食。1例手术效果不佳。随访2年和10年，仅有2例预后良好。结论：FIRES是一种定义明确的严重癫痫综合征，可能属于癫痫性脑病组，其特征是病因不明的新皮质区域发生局灶性或多灶性癫痫发作。免疫球蛋白和生酮饮食可能被认为是潜在有效的治疗方法。(C) 2013年英国癫痫协会。Elsevier Ltd.出版。版权所有。
Purpose: To analyze the electroclinical features, neuroimaging findings, treatment, and outcome of 12 patients with febrile infection-related epilepsy syndrome (FIRES).Methods: This is a retrospective study of 12 children with FIRES with a mean time of follow-up of 6.5 years carried out at the Garrahan Hospital of Buenos Aires between 1997 and 2012.Results: Eight males and four females had focal status epilepticus preceded by febrile infection with a mean age at presentation of 8.5 years. In the acute period, the treatment included antiepileptic drugs (AEDs) in all cases, immunotherapy in 10 cases, and burst-suppression coma in eight. The ketogenic diet was tried in two, plasmapheresis in one, and rituximab in one. Two patients treated with IVIG and one patient given steroids had a good response, but in this phase only three patients had a prolonged good response to IVIG and a ketogenic diet. No patients died in this period. In the chronic epilepsy phase, all children had seizures arising from neocortical regions. All patients had refractory epilepsy, and most mental retardation, and behavioral disturbances. All received different AEDs and in this phase a third patient was put on a ketogenic diet. One patient was operated without good results. Only two cases had a good outcome after 2 and 10 years of follow-up.Conclusion: FIRES is a well-defined severe epileptic syndrome, probably in the group of epileptic encephalopathies, characterized by focal or multifocal seizures arising from the neocortical regions with an unknown etiology. Immunoglobulin and the ketogenic diet may be considered a potentially efficacious treatment. (C) 2013 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.