JAMA Neurol:髓鞘少突胶质细胞糖蛋白抗体与获得性脱髓鞘综合征

2019-09-24 MedSci MedSci原创

髓鞘少突胶质细胞糖蛋白抗体在获得性脱髓鞘综合征患儿中常见,约半数病例为暂时性

髓鞘少突胶质细胞糖蛋白(MOG)自身抗体与脱髓鞘疾病的病程密切相关,近日在一个早期前瞻性获得性脱髓鞘儿童队列研究中,研究人员描述一系列抗-MOG抗体血清学、临床和影像学特征。

来自加拿大儿童脱髓鞘疾病患者参与研究,纳入标准包括:(1)中枢神经系统脱髓鞘事件;(2)发病后45天内至少采集1份血清标本;(3)完整的临床资料。在所招募的430名获得性脱髓鞘综合征患者中,274人参加了分析。平均随访4年,评估了抗-MOG抗体的存在,临床、磁共振成像和脑脊液的特征性表现。

在274名参与者中,140名(51.1%)为女性,平均年龄10.8岁(6.2-13.9)。有三分之一的儿童在脱髓鞘事件发生时抗-MOG抗体呈阳性。临床表现包括视神经炎、横脊髓炎和急性播散性脑脊髓炎,占抗-MOG抗体阳性儿童总数的96%。76例抗-MO抗体阳性者中有51例(67%)出现脑损害,但磁共振成像特征随年龄不同而有差异。在49名抗-MOG抗体阳性者中,26人(53%)观察到基线病变的完全缓解。在连续血清分析中,67例(57%)在发病时血清阳性的患者中有38例转为阴性(中位转换时间,1年)。在所有呈抗-MOG抗体阳性的受试者中,24名儿童中有9名(38%)持续血清阳性,38名儿童中有5名(13%)转为血清阴性。

髓鞘少突胶质细胞糖蛋白抗体在获得性脱髓鞘综合征患儿中常见,约半数病例为暂时性。

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    2020-04-16 yinhl1978
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