摘要
目的探讨儿童髓鞘少突胶质细胞糖蛋白( myelin oligodendrocyte glycoprotein,MOG)抗体脑脊髓炎的临床、影像学特征.方法回顾性分析2016年12月至2018年12月南京医科大学附属儿童医院诊断的13例MOG抗体脑脊髓炎患儿的临床、实验室、影像学及随访等资料.结果 13例患儿中男4例,女9例,平均年龄8岁1个月,首发症状包括发热、嗜睡2 例,视力下降5 例,抽搐发作3 例,尿潴留2 例,共济失调2 例.12例头颅MRI异常,大部分表现为广泛性或孤立的皮质下白质病灶,少部分深部灰质核团和脑干受累;3例脊髓MRI异常,主要表现为长节段横贯性脊髓炎;6例视神经MRI异常,表现为病侧视神经或视交叉异常信号;13例血清MOG抗体滴度1: 10~1: 320.所有患儿对糖皮质激素和丙种球蛋白反应良好,治疗后症状均缓解.随访过程中2例复发,表现为视神经炎,添加硫唑嘌呤后随访1~2年未再复发.结论儿童MOG抗体脑脊髓炎临床以视力下降常见,影像以急性播散性脑脊髓炎样改变为主,免疫抑制剂治疗有效,大部分预后良好.
Objective To investigate the clinical and imaging features of myelin oligodendrocyte glycoprotein antibody(MOG)encephalomyelitis in children. Methods The clinical,laboratory finding,imaging and follow-up data of 13 children with MOG encephalomyelitis(MOG-EM)diagnosed by Children's Hospital of Nanjing Medical Univer-sity from December 2016 to December 2018 were retrospectively analyzed. Results Among the 13 children,4 cases were male and 9 cases were female,the median age was 8 years old and 1 month. Symptoms of the first episode included fever,drowsiness in 2 cases,visual acuity in 5 cases,convulsions in 3 cases,urinary retention in 2 cases,and ataxia in 2 cases. Abnormalities were found in 12 cases by the head magnetic resonance imaging(MRI),most of which showed ex-tensive or isolated subcortical white matter lesions,and a few deep gray matter nuclei and brainstem were involved;3 ca-ses of spinal MRI abnormalities,mainly characterized by long segmental transverse myelitis;6 cases optic nerve MRI ab-normalities were found in 6 cases,manifested as disease side optic nerve or optic chiasm abnormal signals;the titer of serum MOG antibody was 1: 10-1: 320 in 13 cases. All children responded well to glucocorticoids and gamma globu-lin,and all symptoms were alleviated after treatment. Two patients had recurrence during the follow-up period,which was characterized by optic neuritis. After azathioprine addition,there was no recurrence after 1 to 2 years of follow-up. Conclusions Children with MOG antibody encephalomyelitis present a decline in visual acuity commonly. The images are mainly acute disseminated encephalomyelitis-like changes,immunosuppressive therapy is effective,generally with a better prognosis.
作者
徐敏
郭虎
何燕
王莉
梁超
丁乐
郑帼
卢孝鹏
张刚
Xu Min;Guo Hu;He Yan;Wang Li;Liang Chao;Ding Le;Zheng Guo;Lu Xiaopeng;Zhang Gang(Department of Neurology,Children′s Hospital of Nanjing Medical University,Nanjing 210008,China)
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2019年第13期997-1001,共5页
Chinese Journal of Applied Clinical Pediatrics
基金
国家自然科学基金(81801292).
