以脊髓损害为首发症状的髓鞘少突胶质细胞糖蛋白抗体相关性疾病(附2例报道)  

Myelin oligodendrocyte glycoprotein antibody-related diseases with onset with spinal cord injury(report of 2cases)

作　　者：肖潇 汤继宏[1] 张兵兵[1] 武银银 徐晨 杨乐天 徐欢 XIAO Xiao;TANG Jihong;ZHANG Bingbing(Department of Neurology,Children's Hospital of Soochow University,Suzhou 215025,China)

机构地区：[1]苏州大学附属儿童医院神经内科,215025

出　　处：《临床神经病学杂志》2024年第4期283-286,共4页Journal of Clinical Neurology

基　　金：苏州市科技计划(医疗卫生科技创新-临床前沿技术)项目(SKY2022007)。

摘　　要：目的 探讨以脊髓损害为首发症状的髓鞘少突胶质细胞糖蛋白抗体(MOG-Ab)相关性疾病(MOGAD)的临床特点及免疫治疗效果。方法 回顾性分析2例脊髓损害为首发症状的MOGAD患儿的临床资料。结果 2例患儿均以双下肢乏力为首发症状,经外周血及CSF MOG-Ab检测、头颅及脊髓神经影像学检查后确诊为以脊髓损害为首发症状的MOGAD。糖皮质激素冲击治疗后临床症状缓解,复查影像学见异常信号范围缩小。出院后随访5月余临床治愈,症状无反复。结论 以脊髓损害为首发症状的MOGAD为少见疾病类型,其早期临床表现可能为颈背腰部不适、逐渐加重的肢体乏力及大小便障碍等,影像学表现可见多节段脊髓异常信号,CSF及外周血可检测出MOG-Ab。早期识别及诊断,并及时应用糖皮质激素冲击治疗有助于快速缓解临床症状、改善预后。Objective To investigate the clinical characteristics and immunotherapy effect of myelin oligodendrocyte glycoprotein antibody(MOG-Ab)-associated disorders(MOGAD)onset with spinal cord injury.Methods Clinical data of 2 children with MOGAD onset with spinal cord injury were retrospectively analyzed.Results The first symptoms of 2 children were both lower limbs fatigue.After testing the full-length MOG-Ab in both serum and CSF and MRI scanning,they were diagnosed as MOGAD onset with spinal cord injury.The clinical symptoms were relieved after hormone impact treatment,and the range of abnormal signals were reduced by reexamination.After discharge,the patient was followed up for more than 5 months,and the clinical symptoms were cured without recurrence.Conclusions MOGAD onset with spinal cord injury is a rare disease type.Its early clinical manifestations may be neck,back and waist discomfort,gradually aggravated limb weakness,urination and defecation dysfunction.Imaging examination shows abnormal signals of multi-segmental spinal cord,and MOG-Ab could be detected in CSF and peripheral blood.Early identification and diagnosis and timely application of hormone impact treatment can help to quickly relieve clinical symptoms and improve prognosis.

关 键 词：髓鞘少突胶质细胞糖蛋白抗体相关性疾病 脊髓损害 治疗 

分 类 号：R744.5[医药卫生—神经病学与精神病学]