儿童髓鞘少突胶质细胞糖蛋白抗体相关脱髓鞘病的临床、实验室及影像特点

Clinical laboratory and imaging features of myelin oligodendrocyte glycoprotein antibody associated demyelinating disease in children

目的探讨儿童髓鞘少突胶质细胞糖蛋白抗体相关的脱髓鞘脑病的临床、实验室及影像特点。方法针对郑州大学附属儿童医院神经内科2018年8月至2020年10月收治的确诊为髓鞘少突胶质细胞糖蛋白抗体相关的中枢脱髓鞘脑病患儿进行观察回顾性研究,描述患儿的临床、实验室及影像特点,并进行总结分析。结果入组共13名患儿,男女比例为:6∶7,首发年龄为6.9岁(1岁45月~13岁),61.5%(8/13例)为单相病程,38.5%(5/13例)为复发病程,46.1%(6/13例)有前驱事件,症状以发热(9/13例)、头痛(6/13例)、呕吐(5/13例)为主,脑脊液脑脊液细胞数波动在2~116×10^6/L,以单核细胞为主,核磁共振以皮层下白质(10/13例),基底节丘脑(8/13例)受累为主;复发患儿均大于6岁,女孩占80%(4/5例),以视物模糊(2/5例)、发热(2/5例)、头痛(2/5例)为主,所有患儿对激素及丙球反应敏感。结论在本研究中临床症状方面患儿以发热、头痛、呕吐症状最为常见,复发病程中以视物模糊、头痛、发热为主要表现;脑脊液符合病毒性脑炎的特点,脑脊液细胞数与髓鞘少突胶质细胞糖蛋白抗体抗体滴度呈正相关;患儿复发与抗体滴度无直接关系,核磁共振异常信号恢复情况不佳是复发或预后较差的一种提示;本病激素及丙种球蛋白治疗后症状明显减轻。

Objective To investigate the clinical, laboratory and imaging features of demyelinating encephalopathy associated with myelin oligodendrocyte glycoprotein antibody in children.Methods To investigate the clinical, laboratory and imaging characteristics of children with central demyelinating encephalopathy associated with myelin oligodendrocyte glycoprotein antibody in our hospital from August 2018 to October 2020.Results The ratio of male to female was 6∶7. The first onset age was 6.9 years old(1 year, 45 months to 13 years old), 61.5%(8/13 cases) had monophasic course, 38.5%(5/13 cases) had recurrent course, and 46.1%(6/13 cases) had prodromal events. The main symptoms were fever(9/13 cases), headache(6/13 cases), vomiting(5/13 cases). The number of CSF cells fluctuated from 2 to 116×10^6/L, mainly monocytes MRI showed that subcortical white matter(10/13 cases) and basal ganglia thalamus(8/13 cases) were mainly involved;recurrent children were more than 6 years old, 80%(4/5 cases) were girls, mainly with blurred vision(2/5 cases), fever(2/5 cases) and headache(2/5 cases). All children were sensitive to hormone and c-globulin reaction.Conclusion In this study, fever, headache and vomiting were the most common clinical symptoms in children, and blurred vision, headache and fever were the main manifestations in the course of recurrence;cerebrospinal fluid was in line with the characteristics of viral encephalitis, and the number of cells in cerebrospinal fluid was positively correlated with antibody titer of myelin oligodendrocyte glycoprotein antibody;there was no direct relationship between recurrence and antibody titer, and MRI was abnormal The poor recovery of No.1 is a sign of recurrence or poor prognosis;the symptoms of the disease were significantly relieved after treatment with hormone and gamma globulin.