【摘要】 目的 探讨发热感染相关性癫痫综合征的临床特点、治疗方法和预后。方法 收集2017年8月至2022年6月郑州大学第一附属医院儿童重症监护病房收治的17例发热感染相关性癫痫综合征患儿的临床资料，对临床特点、治疗方法和预后进行分析。结果 17例发热感染相关性癫痫综合征患儿中男8例，女9例，发病年龄(7.09±1.77)岁，发热至首次抽搐间隔时间(4.18±1.38) d。癫痫发作类型以局灶性发作为主。患儿发病前体健，均在发热后出现了频繁的癫痫发作或癫痫持续状态，伴有意识障碍，急性期均接受激素、丙种球蛋白和多种抗癫痫药物(4～7种)治疗，效果不佳。10例接受大剂量苯巴比妥治疗，7例发作次数减少，但不良反应较多。7例添加生酮饮食治疗，4例有效，1例接受托珠单抗治疗后发作减少。14例患儿使用机械通气，平均机械通气时间15(11.5,25) d,ICU住院时间36(31,63) d，急性期死亡2例。出院后远期随访11例，1例无临床发作，10例为药物难治性癫痫，9例遗留智力障碍。结论 发热感染相关性癫痫综合征是一种原因不明的灾难性癫痫脑病，急性期对多种抗癫痫药物及麻醉剂治疗效果差，大剂量苯巴比妥可明显减少或终止癫痫发作，但不良反应相对多，早期生酮饮食、针对靶标免疫治疗可能有效。慢性期幸存者往往遗留药物难治性癫痫和智力障碍。更多还原

【Abstract】 Objective To investigate the clinical features,treatments and outcomes of febrile infection-related epilepsy syndrome. Methods The clinical datas of 17 children with febrile infection-related epilepsy syndrome admitted to Pediatric Intensive Care Unit of the First Affiliated Hospital of Zhengzhou University from August 2017 to June 2022 were retrospectively analyzed. The clinical manifestations,treatments and outcomes were analyzed. Results Among the 17 children with febrile infection-related epilepsy syndrome,there were 8 males and 9 females. The age at onset was( 7. 09 ± 1. 77) years,and the interval time from fever onset to the first seizure was( 4. 18 ± 1. 38) days. The type of seizures was mainly focal seizures. The children were healthy before the onset of the disease,but they all developed frequent seizures or status epilepticus after fever,accompanied by consciousness disorders. During the acute phase,they all received treatment with steroids,gamma globulin,and multiple antiepileptic drugs( 4-7 types),but the effects were poor. Ten patients were treated with high-dose phenobarbital,seven patients had significant reduction in seizure frequency but more adverse reactions. The ketogenic diet was tried in seven cases,four of which showed improvement,and one case experienced reduced seizures after receiving tocilizumad treatment.Fourteen children need mechanical ventilation and the time on mechanical ventilation was 15( 11. 5,25) days,ICU length of stay was 36( 31,63) days. Two patients died in the acute phase. At follow-up,only one of eleven was seizure-free and the rest of the patients had refractory epilepsy,nine of them with intellectual disability. Conclusion Febrile infection-related epilepsy syndrome is a catastrophic epileptic encephalopathy with a yet undefined etiology. Multiple antiepileptic drugs and anesthetics have poor therapeutic effect. High-dose phenobarbital is effective in reducing or terminating epileptic seizures,but there are more adverses reactions. Early ketogenic diet and targeted immunotherapy may be effective. Survivors often left with refractory epilepsy and intellectual disability during the chronic phase.更多还原