Persisting myelin oligodendrocyte glycoprotein antibodies in aquaporin-4 antibody negative pediatric neuromyelitis optica
Persisting myelin oligodendrocyte glycoprotein antibodies in aquaporin-4 antibody negative pediatric neuromyelitis optica
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DOI：
10.1177/1352458512470310
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发表时间：
2013-07-01
影响因子：
5.8
通讯作者：
Reindl, M.
中科院分区：
文献类型：
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作者：
Rostasy, K.;Mader, S.;Reindl, M.
背景：最近我们发现在水通道蛋白-4 (AQP4)-免疫球蛋白（IgG）血清阴性的儿童和成人明确和高风险视神经脊髓炎（NMO）患者中可发现髓鞘少突胶质细胞糖蛋白（MOG）抗体。目的：本研究的目的是描述AQP4-IgG血清阴性的小儿明确NMO患者MOG-IgG的临床特征和时间动态。方法：本研究纳入了明确的NMO患儿，并作进一步的AQP4和MOG血清抗体细胞检测。本文对这些患者的临床病程、脑脊液及磁共振成像（MRI）研究进行综述。结果：2008年至2012年间，8名符合明确NMO诊断标准的儿童被招募。2例确诊NMO患儿AQP4-IgG检测阳性，MOG-IgG抗体检测阴性。3例患儿AQP4-IgG和MOG-IgG抗体缺失。3例确诊NMO患儿血清MOG-IgG抗体滴度高（1:16 0），但无aqp4导向的体液免疫应答。后三名儿童的血清样本纵向分析显示，随着时间的推移，MOG-IgG滴度持续高。结论：临床症状和MRI表现高度提示NMO，但MOG-IgG抗体滴度高且持续的儿童患者最有可能代表急性脱髓鞘疾病的一个独特亚群，具有重要的临床和治疗意义。
Background:Recently we showed that antibodies to myelin oligodendrocyte glycoprotein (MOG) can be found in aquaporin-4 (AQP4)-immunoglobulin (IgG) seronegative pediatric and adult patients with definite and high-risk neuromyelitis optica (NMO).Objective:The purpose of this study was to describe the clinical characteristics and temporal dynamics of MOG-IgG in AQP4-IgG seronegative pediatric patients presenting with definite NMO.Methods:Children with definite NMO who were referred for further testing of serum antibodies for AQP4 and MOG with a cell-based assay were included in this study. Clinical disease course, cerebrospinal fluid and magnetic resonance imaging (MRI) studies of these patients were reviewed.Results:Between 2008 and 2012 eight children who fulfilled the diagnostic criteria of definite NMO were recruited. Two children with definite NMO tested positive for AQP4-IgG but were negative for MOG-IgG antibodies. Three children had an absence of AQP4-IgG and MOG-IgG antibodies. Three children with definite NMO had high titers of serum MOG-IgG antibodies (1: 160), but no AQP4-directed humoral immune response. Longitudinal analysis of serum samples of the latter three children showed persisting high MOG-IgG titers over time.Conclusion:Pediatric patients presenting with clinical symptoms and MRI findings highly suggestive of NMO but with high and persisting MOG-IgG antibody titers are most likely to represent a distinct subgroup of acute demyelinating diseases with important clinical and therapeutic implications.