托珠单抗成功治疗儿童发热感染相关癫痫综合征1例及文献复习

A case of febrile infection-related epilepsy syndrome in children successfully treated with tocilizumab and literature review

目的报道托珠单抗在1例儿童发热感染相关癫痫综合征(FIRES)患者中的成功应用,为FIRES患儿的治疗提供新思路。方法总结郑州大学附属儿童医院于2021年2月15日收治的1例FIRES患儿诊疗经过及其对托珠单抗的治疗反应,并对患者进行随访和预后评估,同时对托珠单抗治疗儿童FIRES的文献进行复习。结果该患儿5岁起病,经多种抗癫痫药物、麻醉药物、激素、静脉注射免疫球蛋白等免疫治疗及生酮饮食治疗均无效,呈超级难治性癫痫持续状态,因此于病史36 d及43 d各应用1次托珠单抗(每次4 mg/kg)。第2剂托珠单抗治疗10 d后患儿癫痫发作得以控制。随访10个月,患儿癫痫发作控制良好,认知行为及言语功能明显恢复。目前仅检索到了2篇共3例儿童FIRES患者应用托珠单抗治疗的报道,均显示有效,未见明显不良反应。结论FIRES是一种罕见的难治性癫痫综合征,对多种抗癫痫药物甚至麻醉剂耐药,治疗困难,预后差。初步的尝试显示托珠单抗对儿童FIRES患者有效,耐受性好,是一种具备潜力的治疗手段之一。

Objective To report a case of tocilizumab successfully used in a child with febrile infection-associated epilepsy syndrome(FIRES),and to provide a new idea for the treatment of FIRES in children.Methods The diagnosis and treatment of 1 case of FIRES admitted in Children′s Hospital Affiliated to Zhengzhou University on February 15,2021 were described,and the prognosis and follow-up of the child were evaluated.At the same time,the literatures on tocilizumab in the treatment of children′s FIRES were reviewed.Results A 5-year-old case of FIRES was reported.The child was extremely refractory to immunotherapy and anti-seizure medicines,anesthetics and ketogenic diet.So he was treated with tocilizumab(each time 4 mg/kg)at the 36th day and 43rd day,and epileptic seizures were controlled 10 days after the 2nd doses of tocilizumab.During a follow-up of 10 months,his epileptic seizures were controlled and the cognitive behavior and speech function were well recovered.At present,only 3 cases of FIRES in children have been reported all over the world.All the seizures were well controlled and no obvious adverse reactions were observed.Conclusions FIRES is a rare refractory epilepsy syndrome,resistant to many kinds of anti-seizure medicines or even anesthetic agents,which is difficult to treat and has poor prognosis.Preliminary trials have shown that tocilizumab is effective and well tolerated in children with FIRES.It may be a potential therapeutic modality for children with FIRES.