儿童神经副肿瘤综合征11例

Clinical analysis of paraneoplastic neurologic syndromes in children

目的 分析儿童神经副肿瘤综合征(PNS)的临床特点。方法 分析2017年1月至2022年6月河北省儿童医院收治的11例儿童PNS患者的临床特点、影像及实验室检查结果、治疗及预后等。结果 神经母细胞源性肿瘤的PNS患者8例,其中7例接受手术及化疗治疗,4例术后同时免疫治疗,仅1例遗留神经系统后遗症,随访至2022年9月肿瘤均无复发;畸胎瘤抗N-甲基-D-天冬氨酸(NMDA)受体脑炎患者3例,2例成熟性畸胎瘤,1例囊实性未成熟性畸胎瘤,均接受手术联合免疫治疗,随访至2022年9月均无复发。结论 以共济失调、眼震起病的0-3岁小年龄组的PNS患者应首先考虑神经母细胞源性肿瘤,以抗NMDA受体脑炎起病的女性儿童,需警惕畸胎瘤。对于肿瘤筛查阴性的患者长期随访、定期筛查是必要的。早期发现及治疗原发肿瘤是决定预后的重要因素。

Objective The clinical characteristics of children paraneoplastic neurologic syndromes(PNS)were analyzed.Methods The clinical characteristics,imaging and laboratory results,treatment and prognosis of11 children with PNS admitted to Hebei Children's Hospital from January 2017 to June 2022 were retrospectively analyzed.Results There were 8 patients with PNS complicated with neuroblastic tumor,among which 7 patients received surgery and chemotherapy,4 patients received immunotherapy at the same time after surgery,and only 1patient had neurological sequelae.No recurrence of the tumor has occurred since the follow-up.Three patients with anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis complicated with teratoma,including 2 mature teratoma and 1 cystic solid immature teratoma,were treated with surgery combined with immunotherapy,and no recurrence had occurred so far.Conclusion Patients with PNS in the young age group of 0-3 years,with ataxia and nystagmus onset,should first be considered for tumors of neuroblastic.Teratoma should be warned in female children with anti-NMDA receptor encephalitis onset.Long-term follow-up and regular screening are necessary for patients with negative tumor screening.Early detection and treatment of primary tumors are important prognostic factors.