Institute of Clinical Neuroimmunology (M.S., L.A.G., M.K., F.S.T., E.S., R.H., E.M., T.K.), Department of Radiology (B.E.-W.), and Laboratory for Immunogenetics (A.D.), Medical Campus Großhadern, Ludwig-Maximilians-Universität München; Department of Neurology (M.K.), Hertie Institut für klinische Hirnforschung, Universitätsklinikum Tübingen; Department of Neuropathology (I.M., W.B.), University Medical Center, Georg August University, Göttingen; Department of Paediatric Neurology and Developmental Medicine (A.B.), Dr. von Hauner Children's Hospital, Ludwig-Maximilians-Universität München; and Munich Cluster for Systems Neurology (SyNergy) (R.H.), Munich, Germany.
Neurol Neuroimmunol Neuroinflamm. 2016 Jun 30;3(5):e257. doi: 10.1212/NXI.0000000000000257. eCollection 2016 Oct.
To evaluate the presence of antibodies to conformation-intact myelin oligodendrocyte glycoprotein (MOG) in a subgroup of adult patients with clinically definite multiple sclerosis (MS) preselected for a specific clinical phenotype including severe spinal cord, optic nerve, and brainstem involvement.
Antibodies to MOG were investigated using a cell-based assay in 3 groups of patients: 104 preselected patients with MS (group 1), 55 age- and sex-matched, otherwise unselected patients with MS (group 2), and in 22 brain-biopsied patients with demyelinating diseases of the CNS (n = 19 with MS), 4 of whom classified as MS type II (group 3). Recognized epitopes were identified with mutated variants of MOG.
Antibodies to MOG were found in about 5% (5/104) of preselected adult patients with MS. In contrast, in groups 2 and 3, none of the patients tested positive for MOG antibodies. Patients with MS with antibodies to MOG predominantly manifested with concomitant severe brainstem and spinal cord involvement and had a severe disease course with high relapse rates and failure to several disease-modifying therapies. Three of them had been treated with plasma exchange with a favorable response. All anti-MOG-positive patients with MS showed typical MS lesions on brain MRI. Longitudinal analysis up to 9 years revealed fluctuations and reappearance of anti-MOG reactivity. Epitope mapping indicated interindividual heterogeneity, yet intraindividual stability of the antibody response.
Antibodies to MOG can be found in a distinct subgroup of adult MS with a specific clinical phenotype and may indicate disease heterogeneity.
评估特定临床表型（包括严重脊髓、视神经和脑干受累）的成年临床确诊多发性硬化（MS）患者亚组中是否存在针对完整髓鞘少突胶质细胞糖蛋白（MOG）的抗体。
使用基于细胞的测定法，在 3 组患者中研究了针对 MOG 的抗体：104 名预先选择的 MS 患者（第 1 组），55 名年龄和性别匹配、未选择的 MS 患者（第 2 组），以及 22 名接受过脑活检的脱髓鞘性中枢神经系统疾病患者（n = 19 例 MS，其中 4 例为 MS 型 II 型）（第 3 组）。用 MOG 的突变变体鉴定了识别的表位。
在大约 5%（5/104）的预先选择的成年 MS 患者中发现了针对 MOG 的抗体。相比之下，在第 2 组和第 3 组中，未发现任何患者对 MOG 抗体呈阳性。具有 MOG 抗体的 MS 患者主要表现为同时伴有严重的脑干和脊髓受累，且疾病过程严重，复发率高，对几种疾病修饰疗法均无效。其中 3 例接受血浆交换治疗，反应良好。所有 MS 伴 MOG 阳性的患者在脑 MRI 上均显示出典型的 MS 病变。长达 9 年的纵向分析显示出抗 MOG 反应的波动和再现。表位映射表明个体间存在异质性，但抗体反应具有个体内稳定性。
在具有特定临床表型的成年 MS 中，可能会发现针对 MOG 的抗体，这可能表明疾病异质性。
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