Alparslan Caner, Kamit-Can Fulya, Anıl Ayşe Berna, Olgaç-Dündar Nihal, Çavuşoğlu Dilek, Göç Zeynep
Department of Pediatrics, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Turkey.
Department of Pediatric Intensive Care Unit, University of Health Sciences, Tepecik Training and Research Hospital, Izmir, Turkey.
Turk J Pediatr. 2017;59(4):463-466. doi: 10.24953/turkjped.2017.04.014.
Alparslan C, Kamit-Can F, Anıl AB, Olgaç-Dündar N, Çavuşoğlu D, Göç Z. Febrile infection-related epilepsy syndrome (FIRES) treated with immunomodulation in an 8-year-old boy and review of the literature. Turk J Pediatr 2017; 59: 463-466. Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epilepsy syndrome which is characterized by acute onset of refractory status epilepticus following a febrile infection occurring in previously normal children. Despite the various treatment options that have been tried, exact treatment strategy is still undetermined. This is the first pediatric case of FIRES from Turkey which was successfully treated with intravenous immunoglobulin (IVIG). A previously healthy 8-year-old boy was referred to our hospital with a pre-diagnosis of status epilepticus and encephalitis. He presented with acute onset of convulsions and unconsciousness following fever and malaise lasting 7 days. On physical examination Glasgow coma scale was 12, his pupils were miotic. He had cafe-au-lait spots on his body. His fundus examination, cerebrospinal fluid findings and cranial magnetic resonance imaging did not reveal any abnormality. Results of comprehensive search for metabolic, toxicological, infectious and autoimmune etiologies were all negative. Generalized slowing was seen on the electroencephalography (EEG) of the patient indicating possible encephalopathy. The patient developed convulsive status epilepticus and was intubated on day 5. His seizures were controlled by continuous infusion of midazolam, thiopental and used for 4 days. Phenytoin, levetiracetam, topiramate were used simultaneously. IVIG was administered as an immunomodulator for refractory seizures on day-9. The patient was extubated on day 11. The diagnosis was made after a comprehensive negative search for central nervous system infection, autoimmune and metabolic diseases. At follow up it was learnt that he had had only two seizures in two years. Status epilepticus did not recur. Clinicians should keep in mind FIRES which is a diagnosis of exclusion especially in refractory status epilepticus. IVIG treatment could have a benefit in these patients.
阿尔帕斯兰·C、卡米特-坎·F、阿尼尔·AB、奥尔加奇-邓达尔·N、恰武索卢·D、戈奇·Z。一名8岁男孩经免疫调节治疗的发热感染相关性癫痫综合征（FIRES）及文献复习。《土耳其儿科学杂志》2017年；59：463 - 466。发热感染相关性癫痫综合征（FIRES）是一种灾难性癫痫综合征，其特征为先前正常的儿童在发热感染后急性发作难治性癫痫持续状态。尽管尝试了各种治疗方案，但确切的治疗策略仍未确定。这是土耳其首例成功接受静脉注射免疫球蛋白（IVIG）治疗的FIRES儿科病例。一名此前健康的8岁男孩因癫痫持续状态和脑炎的初步诊断被转诊至我院。他在发热和不适持续7天后出现惊厥和意识丧失的急性发作。体格检查时格拉斯哥昏迷量表评分为12分，瞳孔缩小。他身上有咖啡牛奶斑。眼底检查、脑脊液检查结果及头颅磁共振成像均未发现任何异常。对代谢、毒理学、感染和自身免疫病因进行全面检查的结果均为阴性。患者脑电图（EEG）显示广泛性减慢，提示可能存在脑病。患者在第5天出现惊厥性癫痫持续状态并插管。通过持续输注咪达唑仑、硫喷妥钠控制其癫痫发作，用药4天。同时使用苯妥英钠、左乙拉西坦、托吡酯。在第9天给予IVIG作为难治性癫痫发作的免疫调节剂。患者在第11天拔管。在对中枢神经系统感染、自身免疫和代谢疾病进行全面阴性检查后做出诊断。随访得知他在两年内仅发作过两次癫痫。癫痫持续状态未复发。临床医生应牢记FIRES，尤其是在难治性癫痫持续状态时，它是一种排除性诊断。IVIG治疗可能对这些患者有益。
