临床儿科杂志 ›› 2021, Vol. 39 ›› Issue (11): 865-.doi: 10.3969/j.issn.1000-3606.2021.11.017

• 综合报道 • 上一篇    下一篇

糖皮质激素治疗抗N- 甲基-D- 天冬氨酸受体脑炎致硬膜外脂肪增多症1 例报告并文献复习

肖慧媚, 李碧云, 裴铮, 韩鹏慧, 常燕群   

  1. 广东省妇幼保健院(广东广州 511400)
  • 发布日期:2021-11-19
  • 通讯作者: 常燕群 电子信箱:cyq 01 @sina.com

Spinal epidural lipomatosis caused by glucocorticoids in the treatment of children anti-N-methyl-Daspartate receptor encephalitis: a case report and literature review

XIAO Huimei, LI Biyun, PEI Zheng, HAN Penghui, CHANG Yanqun   

  1. Guangdong Women and Children Hospital, Guangzhou 511400 , Guangdong, China
  • Published:2021-11-19

摘要: 目的 探讨糖皮质激素治疗抗N-甲基-D-天冬氨酸受体脑炎(抗NMDAR脑炎)致硬膜外脂肪增多症(SEL) 的临床特征。方法 回顾分析1例抗NMDAR脑炎致SEL患儿的临床资料。检索万方数据库及PubMed数据库,复习相关 文献。结果 患儿,女,8岁,表现为右手不自主活动、癫痫样发作伴精神行为异常,脑脊液抗谷氨酸受体抗体IgG 1:32,诊 断为抗NMDAR脑炎。患儿先后接受丙种球蛋白、糖皮质激素(泼尼松)、血浆置换及环磷酰胺免疫治疗。随访中发现患儿 脑脊液蛋白异常升高,脊髓磁共振(MRI)示脊髓SEL。逐渐减停泼尼松,以合理饮食及运动控制体质量后,复查脊髓MRI 明显好转。检索文献,国外有14篇文献报道15例类固醇激素治疗其他疾病致SEL。儿童SEL可出现背痛、无力、急性麻痹 等临床症状,也可无症状。结论 外源性类固醇激素治疗抗NMDAR脑炎引起的儿童SEL,可在临床症状出现之前早期诊 断,并通过减停激素及控制体质量等治疗完全康复。

关键词: 硬膜外脂肪增多症; 抗NMDAR脑炎; 糖皮质激素

Abstract: Objective To investigate clinical characteristics and treatment of spinal epidural lipomatosis (SEL) caused by glucocorticoids in the treatment of anti-N-methyl-D- aspartate receptor (NMDAR) encephalitis. Methods The clinical data of one child diagnosed as anti-NMDAR encephalitis in May 2018 were retrospectively analyzed. A literature search was conducted at Wanfang database and PubMed, and the relevant literature were reviewed. Results The 8 -year-old girl was hospitalized for "involuntary movement of the right limb, seizure and abnormal mental behavior". The anti-glutamate receptor antibody IgG of CSF was positive with a titer of 1 :32 . Then the diagnose of anti-NMDAR encephalitis was made. She was treated with gamma globulin, glucocorticoid, prednisone, plasma exchange and cyclophosphamide immunotherapy. During the follow-up, abnormal increase of cerebrospinal fluid protein was found. Spinal cord MRI showed spinal epidural lipomatosis. We then stop prednisone by dose tapering and help her lose weight through diet control and exercise. Then the follow-up spinal MRI showed significant improvement. At present, there are 14 English papers published about SEL caused by steroid therapy in the treatment of other childhood diseases, but no report about SEL caused by glucocorticoids in the treatment of children with NMDAR encephalitis. Children with SEL may present back pain, numbness, weakness, abnormal gait, incontinence and acute paralysis and in some cases may present no symptom. Conclusion The use of steroids for treating anti-NMDAR encephalitis may caused SEL, which could be early diagnosed before the clinical symptoms occur and treated by stopping steroids and controlling body weight.

Key words: Spinal epidural lipomatosis; Anti-N-methyl-d-aspartate receptor encephalitis; Glucocorticoid