目的 分析儿童抗N-甲基-D-天冬氨酸受体(anti-N-methyl-D-aspartate-receptor, NMDAR)脑炎合并横纹 肌溶解症(rhabdomyolysis, RM)的临床特点及预后。方法 回顾分析4例抗NMDAR脑炎合并RM患儿的临床资料。结 果 患儿平均起病年龄为(9.3±4.3)岁,男3例、女1例,出现首发症状至确诊为抗NMDAR脑炎的中位时间为14(10~20)天, 出现首发症状至发展为RM的中位时间为29(22 ~ 40)天。RM的诱因包括感染及癫痫持续状态(3例),持续不自主运动(1 例)。4例患儿中1例患儿接受糖皮质激素、静脉输注人免疫球蛋白及水化碱化治疗后恢复好;其余3例合并感染及癫痫持 续状态者对上述治疗反应差,接受血浆置换及持续血液滤过治疗,1例死于脓毒性休克,另外2例最终接受利妥昔单抗治疗, 1例有效,1例遗留严重神经系统后遗症。结论 儿童抗NMDAR脑炎可合并RM,危重患者可能对一线免疫治疗及水化碱 化治疗反应不佳,应尽早启动血液净化治疗。
To explore the clinical features and prognosis of children with anti-N-methyl-D-aspartatereceptor (NMDAR) encephalitis complicated with rhabdomyolysis (RM). Methods The clinical data of 4 children with anti-NMDAR encephalitis complicated with RM were retrospectively analyzed. Results There were 3 boys and 1 girl with an average onset age of ( 9 . 3 ±4 . 3 ) years. The median time between onset symptom and diagnosis of anti-NMDAR encephalitis was 14 ( 10 - 20 ) days, and the median time between onset symptom and development of RM was 29 ( 22 - 40 ) days. The causes of RM included infection and status epilepticus ( 3 cases), and persistent involuntary movement ( 1 case). One patient recovered well after receiving steroid and intravenous immunoglobulin treatment and hydration and alkalization therapy. The other 3 patients with complicated infection and status epilepticus had poor response to the treatment and received plasma exchange and continuous hemofiltration. One died of septic shock, and 2 received rituximab treatment. One recovered well, while the other one had severe neurological sequelae. Conclusion Children with anti-NMDAR encephalitis can be complicated with RM. Patients in critical condition may have poor response to first-line immunotherapy and hydration and alkalization therapy, and blood purification therapy should be initiated in time.