Lancet Neurol:儿童髓鞘少突胶质糖蛋白抗体阳性的疾病特征

2020-02-11 MedSci MedSci原创

研究发现,MOG抗体阳性儿童患者的疾病谱更广,包括脱髓鞘综合征和脑炎

成年人髓鞘少突胶质糖蛋白(MOG)抗体阳性患者主要为脱髓鞘综合征患者,但对于儿童MOG抗体相关综合征的研究尚不充分。近日研究人员就MOG抗体阳性在儿科脱髓鞘和脑炎综合征的频率和分布进行了考察。

急性播散性脑脊髓炎(ADEM)以外的脱髓鞘综合征和脑炎患儿参与研究,患儿进行MOG抗体检测,对阳性患者进行症状、治疗、治疗响应、预后以及与不良预后相关的表型研究。

A组中包含239名脱髓鞘综合征患儿,B组中包含296名非ADEM脑炎患儿,116名患儿MOG阳性,其中A组94例,B组22例(39 vs 7%)。阳性患儿平均年龄6.2岁,女童占49%。116例患者的综合征包括ADEM(46 [68%])、非ADEM脑炎(22[19%])、视神经炎(20[17%])、脊髓炎(13[11%])、视神经脊髓炎谱系障碍(6[5%])和其他疾病(9[8%])。研究随访42个月,116名患者中,33人复发(28%)。86%的患者在初次诊断后接受了类固醇、静脉注射免疫球蛋白或血浆交换治疗,97%的复发患者接受了上述治疗。33%的复发患者接受了利妥昔单抗治疗。116名患者中,99人(85%)康复(mRS <2),而17人出现中重度缺陷((mRS >2,1人死亡)。预后不良的表型包括退化性复发进展为白质营养不良样特征,广泛的皮质性脑炎演变为萎缩。复发患者接受治疗后MOG阴性需要更长的时间(HR=0.18)。

研究发现,MOG抗体阳性儿童患者的疾病谱更广,包括脱髓鞘综合征和脑炎。

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    2020-04-10 yinhl1978
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    2020-11-20 howi
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