热性感染相关性癫痫综合征1例报道

Febrile Infection-Related Epilepsy Syndrome: A Case Report

摘要: 目的：探讨热性感染相关性癫痫综合征(febrile infection-related epilepsy syndrome, FIRES)的临床特点和诊疗对策。方法：对我院收治的1例FIRES患儿的临床资料进行回顾性分析。结果：患儿以精神行为异常起病，起病前有发热感染病史，病程中出现难治性部分性癫痫持续状态(refractory partial status epilepticus, RPSE)，经经验性抗中枢神经系统感染治疗及多种抗癫痫药物(antiepileptie drugs, AEDs)联合治疗2月，发作未控制，家属放弃治疗出院，出院后20天患儿死于癫痫持续状态(SE)。结论：FIRES临床中少见，目前治疗困难，应注意早期识别，尽早予以大剂量抗癫痫药物(AEDs)抗癫痫和(或)生酮饮食(ketogenic diet, KD)治疗，积极控制抽搐，可能减轻脑损伤并改善预后。

Abstract: Objective: To explore the clinical features, diagnosis and treatment of febrile infection-related epilepsy syndrome (FIRES). Methods: The clinical data of one case with FIRES admitted to our hospital were retrospectively analyzed. Results: The child presented with mental and behavior disorder and refractory partial status epilepticus who had a febrile illness antecedent. The symptoms of the child didn’t improve after the treatment of anti-central nervous system infection empirically and antiepileptie drugs for 2 months. And then the therapy of the child was withdrew and he died of status epilepticus after 20 days. Conclusion: The FIRES is rare and the treatment is very difficult at present.Early recognition is of paramount importance to start treatment in an early stage. It should be given high dose of antiepileptic drugs or ketogenic diet, or both, as soon as possible. Maybe it could reduce brain damage and improve the prognosis.