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目的总结热性感染相关性癫痫综合征(FIRES)的临床表现、脑电图及头颅MRI特征,探讨其发病机制及目前治疗进展。方法分析武汉儿童医院神经内科收治1例FIRES患儿的临床表现、脑电图、头颅MRI特征,复习文献资料,总结其临床、脑电图、头颅MRI特征及诊治进展。结果患儿为学龄期男性儿童,急骤起病,表现为发热、意识障碍及药物难治性的癫痫持续状态。脑电图显示背景为弥漫性慢波活动,监测到左侧额极、前颞区起始的部分性发作。头颅MRI信号未见异常。经生酮饮食(KD)治疗3天后,发作较前明显减少并转入普通病房,出院后继续予KD及抗癫痫药物治疗,1个月后复诊,患儿偶有惊厥发作,意识较前好转,智能较发病前明显减退。文献显示该病主要发生在既往健康的学龄期儿童,常由发热诱导,继而出现难治性癫痫持续状态或丛集样癫痫发作。FIRES预后不良,幸存者多遗留药物难治性癫痫和严重的认知障碍,普遍认为KD对FIRES患者疗效显著。结论FIRES的病因不明,可能由免疫介导所致。好发于学龄期儿童,常由发热诱导,癫痫发作呈暴发性、药物难治性,KD对控制急性期癫痫发作及改善预后有效。
Abstract：Objective To summarize the clinical manifestations, electroencephalogram and MRI features of febrile infection-related epilepsy syndrome(FIRES),and to explore its pathogenesis and current treatment progress. Methods The clinical manifestations,EEG and head MRI features of a child with FIRES in neurology department of Wuhan Children′s Hospital were analyzed. The literatures were reviewed;the clinical,EEG,head MRI features,and diagnosis and treatment progress were summarized. Results The child was school-age male with acute onset,who showed fever,disturbance of consciousness and drug-refractory status epilepticus. The EEG showed that the background was diffuse slow wave activity. In EEG,the partial episodes of left frontal and anterior temporal regions were monitored. There was no abnormality in the MRI signal of the brain. After three days of treatment with ketogenic diet(KD),the seizure was significantly reduced and the patient was transferred to the general ward. After discharge,KD and anti-epileptic drugs were continued. After one month,the return visit showed the child was occasionally convulsed and the consciousness improved,intelligence was significantly lower than that before onset. The literature shows that the disease mainly occurs in previously healthy school-age children,often induced by fever,followed by refractory status epilepticus or cluster-like seizures. FIRES has a poor prognosis. Survivors often have drug-refractory epilepsy and severe cognitive impairment. KD is generally considered to have a significant effect on patients with FIRES. Conclusion The cause of FIRES is unknown and may be caused by immune mediation. FIRES always occurs in school-age children,often induced by fever,its seizures are fulminant,it is drug-refractory,KD is effective in controlling acute seizures and improving prognosis.
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基本信息:
DOI：10.16389/j.cnki.cn42-1737/n.2019.05.012
中图分类号:R742.1
引用信息:
[1]杨伟明,孙丹.热性感染相关性癫痫综合征1例病例报道并文献复习[J].江汉大学学报(自然科学版),2019,47(05):459-463.DOI:10.16389/j.cnki.cn42-1737/n.2019.05.012.
2019-10-08
2019-10-08
2019-10-08