Dong Xueshan, Jiang Yan, Yuan Ping, Fan Xiao, Ma Jiannan, Wu Peng, Jiang Li, Li Xiujuan
Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China.
China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing, China.
Front Neurosci. 2023 May 19;17:1128422. doi: 10.3389/fnins.2023.1128422. eCollection 2023.
This study aimed to compare the clinical, radiological, therapeutic, and prognostic differences between pediatric patients showing acute disseminated encephalomyelitis (ADEM) with and without myelin oligodendrocyte glycoprotein (MOG) antibodies.
We retrospectively collected all available data of children diagnosed with ADEM and tested for serum MOG antibodies at the Children's Hospital of Chongqing Medical University from January 2017 to May 2021.
A total of 62 patients were included in our cohort, of which 35 were MOG-seropositive and 27 were MOG-seronegative. MOG-seropositive ADEM children presented with significantly lower rates of seizures ( = 0.038) and cranial nerve (III-XII) palsy ( = 0.003). Isolated leukocytosis in the blood was more common in ADEM children with MOG antibodies ( < 0.001). The two groups showed no significant differences in the distributions and extent of the MRI lesions as well as the appearance of typical/atypical magnetic resonance imaging (MRI) features. MOG-seropositive children were more likely to relapse ( = 0.017) despite having slower oral prednisolone tapering after acute treatments ( = 0.028). In scoring performed on the basis of two neurological function scoring systems, MOG-seropositive children showed milder neurological disability status at onset ( = 0.017 and 0.025, respectively) but showed no difference during follow-up.
In summary, the differences in the clinical manifestations and auxiliary examination findings for MOG-seropositive and MOG-seronegative ADEM children lacked significance and specificity, making early identification difficult. MOG-seropositive children were more likely to relapse and showed slower steroid tapering. Moreover, MOG-seronegative children tended to have more severe neurological impairments at onset with no difference during follow-up.
本研究旨在比较伴有和不伴有髓鞘少突胶质细胞糖蛋白（MOG）抗体的急性播散性脑脊髓炎（ADEM）患儿在临床、影像学、治疗及预后方面的差异。
我们回顾性收集了2017年1月至2021年5月在重庆医科大学附属儿童医院被诊断为ADEM并检测血清MOG抗体的所有患儿的可用数据。
我们的队列共纳入62例患者，其中35例MOG血清阳性，27例MOG血清阴性。MOG血清阳性的ADEM患儿癫痫发作率（P = 0.038）和脑神经（III - XII）麻痹发生率（P = 0.003）显著更低。血液中单纯白细胞增多在伴有MOG抗体的ADEM患儿中更常见（P < 0.001）。两组在MRI病变的分布和范围以及典型/非典型磁共振成像（MRI）特征的表现方面无显著差异。MOG血清阳性患儿尽管在急性治疗后口服泼尼松龙减量较慢（P = 0.028），但更易复发（P = 0.017）。在基于两种神经功能评分系统进行的评分中，MOG血清阳性患儿起病时神经功能残疾状态较轻（分别为P = 0.017和0.025），但随访期间无差异。
总之，MOG血清阳性和血清阴性的ADEM患儿在临床表现和辅助检查结果上的差异缺乏显著性和特异性，难以早期识别。MOG血清阳性患儿更易复发且激素减量较慢。此外，MOG血清阴性患儿起病时神经功能损害往往更严重，随访期间无差异。
