Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, Guangzhou, Guangdong, China.
Front Immunol. 2023 Jun 2;14:1162647. doi: 10.3389/fimmu.2023.1162647. eCollection 2023.
To study the clinical features of children diagnosed with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in southern China.
Clinical data of children diagnosed with MOGAD from April 2014 to September 2021 were analyzed.
A total of 93 children (M/F=45/48; median onset age=6.0 y) with MOGAD were involved. Seizures or limb paralysis was the most common onset or course symptom, respectively. The most common lesion locations in brain MRI, orbital MRI, and spinal cord MRI were basal ganglia and subcortical white matter, the orbital segment of the optic nerve, and the cervical segment, respectively. ADEM (58.10%) was the most common clinical phenotype. The relapse rate was 24.7%. Compared with the patients without relapse, relapsed patients had a longer interval from onset to diagnosis (median: 19 days VS 20 days) and higher MOG antibody titer at onset (median: 1:32 VS 1:100) with longer positively persistent (median: 3 months VS 24 months). All patients received IVMP plus IVIG at the acute phase, and 96.8% of patients achieved remission after one to three courses of treatment. MMF, monthly IVIG, and maintaining a low dose of oral prednisone were used alone or in combination as maintenance immunotherapy for relapsed patients and effectively reduced relapse. It transpired 41.9% of patients had neurological sequelae, with movement disorder being the most common. Compared with patients without sequelae, patients with sequelae had higher MOG antibody titer at onset (median: 1:32 VS 1:100) with longer persistence (median: 3 months VS 6 months) and higher disease relapse rate (14.8% VS 38.5%).
Results showed the following about pediatric MOGAD in southern China: the median onset age was 6.0 years, with no obvious sex distribution difference; seizure or limb paralysis, respectively, are the most common onset or course symptom; the lesions of basal ganglia, subcortical white matter, the orbital segment of the optic nerve, and cervical segment were commonly involved in the CNS MRI; ADEM was the most common clinical phenotype; most had a good response to immunotherapy; although the relapse rate was relatively high, MMF, monthly IVIG and a low dose of oral prednisone might effectively reduce relapse; neurological sequelae were common, and possibly associated with MOG antibody status and disease relapse.
研究中国南方地区诊断为髓鞘少突胶质细胞糖蛋白抗体相关性疾病（MOGAD）患儿的临床特征。
分析 2014 年 4 月至 2021 年 9 月期间诊断为 MOGAD 的儿童的临床资料。
共纳入 93 例 MOGAD 患儿（M/F=45/48；中位发病年龄=6.0 岁）。以癫痫发作或肢体瘫痪为最常见的起病或病程症状。脑 MRI、眼眶 MRI 和脊髓 MRI 最常见的病变部位分别为基底节和皮质下白质、视神经眶段和颈段。脱髓鞘疾病样表现（ADEM）（58.10%）是最常见的临床表型。复发率为 24.7%。与无复发患者相比，复发患者的发病至确诊时间间隔更长（中位数：19 天 VS 20 天），发病时 MOG 抗体滴度更高（中位数：1:32 VS 1:100），且阳性持续时间更长（中位数：3 个月 VS 24 个月）。所有患者在急性期均接受 IVMP+IVIG 治疗，96.8%的患者经 1-3 个疗程治疗后达到缓解。MMF、每月 IVIG 和维持低剂量口服泼尼松单独或联合作为复发患者的维持免疫治疗，有效降低了复发率。41.9%的患者出现神经后遗症，以运动障碍最为常见。与无后遗症患者相比，有后遗症患者发病时 MOG 抗体滴度更高（中位数：1:32 VS 1:100），阳性持续时间更长（中位数：3 个月 VS 6 个月），疾病复发率更高（14.8% VS 38.5%）。
结果显示，中国南方地区儿童 MOGAD 具有以下特点：中位发病年龄为 6.0 岁，无明显性别分布差异；以癫痫发作或肢体瘫痪为最常见的起病或病程症状；CNS MRI 常见基底节、皮质下白质、视神经眶段和颈段受累；ADEM 是最常见的临床表型；多数患者对免疫治疗反应良好；尽管复发率相对较高，但 MMF、每月 IVIG 和低剂量口服泼尼松可能有效降低复发率；神经后遗症常见，可能与 MOG 抗体状态和疾病复发有关。
