Objective: To study the clinical features and treatment outcome of children with myelin oligodendrocyte glycoprotein (MOG) antibody-associated disorders (MOGAD).
Methods: A retrospective analysis was performed for the clinical data of 28 children with MOGAD (with 38 demyelinating episodes).
Results: Among the disease spectrums of 28 children with MOGAD, optic neuritis was the most common (12 cases, 43%), followed by acute disseminated encephalomyelitis (9 cases, 32%). Among the 38 demyelinating episodes in the 28 children, there were 29 cases (76%) of lesions in the acute stage on head magnetic resonance imaging (MRI), and most of these lesions were extensive or isolated subcortical white matter lesions. A total of 24 cases of spinal MRI results in the acute stage were recorded, among which there were 11 cases (46%) of spinal lesions. MRI abnormalities of the optic nerve were found in 18 cases of optic neuritis in the acute stage. Of the 28 children, 20 (71%) had an increase in white blood cell count in cerebrospinal fluid, with lymphocytes as the most common type of cells, and 3 children had an increase in protein. The titer of serum MOG antibody was 1:10-1:320 in the 28 children. All 28 children were administered with glucocorticoids, along with immunoglobulin in 18 children. The symptoms of 26 children (93%) were alleviated during follow-up, and only 2 children had neurological sequela of the optic function.
Conclusions: The clinical manifestations are diverse in children with MOGAD. Immunotherapy is effective and most children have a good prognosis.
目的: 探讨儿童髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病（MOGAD）患儿的临床特点及治疗转归。
方法: 回顾性分析28例MOGAD患儿（38例次脱髓鞘发作）的临床资料。
结果: 28例MOGAD患儿的疾病谱中，视神经炎占比最高（12例，43%），其次为急性播散性脑脊髓炎（9例，32%）。28例患儿（38例次脱髓鞘发作）急性期头颅MRI显示病变者共29例次（76%），大部分表现为广泛性或孤立性皮质下白质病灶。共记录24例次急性发作期脊髓MRI结果，其中脊髓病变者11例次（46%）。18例次视神经炎急性期MRI显示均伴视神经病变。20例（71%）患儿脑脊液白细胞升高，细胞分类以淋巴细胞为主，蛋白升高者3例。28例患儿血清MOG抗体滴度1：10~1：320。28例患儿均给予了糖皮质激素治疗，18例同时予以免疫球蛋白治疗。随访中26例（93%）治疗后症状基本缓解，仅2例遗留视觉能区的神经系统后遗症。
结论: MOGAD儿童的临床表现呈多样性；免疫治疗有效，大部分预后良好。