Department of Neurology (S.-M.K., J.-S.K., K.S.P., K.-W.L.) and Ophthalmology (S.-J.K.), Seoul National University College of Medicine, Seoul, Republic of Korea; Nuffield Department of Clinical Neurosciences (M.R.W., A.V., P.W.), John Radcliffe Hospital, Oxford, United Kingdom; and Department of Neurology (K.S.P.), Seoul National University Bundang Hospital, Gyeonggi, Republic of Korea.
Neurol Neuroimmunol Neuroinflamm. 2015 Oct 15;2(6):e163. doi: 10.1212/NXI.0000000000000163. eCollection 2015 Dec.
To evaluate the clinical relevance of myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in a cohort of adults with inflammatory demyelinating disease (IDD) of the CNS.
Live cell-based assays for MOG-Ab (IgG1 subset) and antibody to aquaporin-4 (AQP4-Ab) were performed in a cohort of 270 adult patients with IDD and 72 controls. Patients were first grouped by positive antibody result as MOG-Ab or AQP4-Ab, and the remainder were grouped by published diagnostic criteria.
Seventeen patients with IDD (6.3%) had MOG-Abs and 49 patients (18.1%) had AQP4-Abs; none had both antibodies. The MOG-Ab patients predominantly manifested with isolated symptoms of optic neuritis (83%). One-third of these patients experienced relapses, which involved only the optic nerve, and all relapsed within 1 year of disease onset. At onset, MRI in the MOG-Ab group uniquely demonstrated perineural enhancement, extending to the soft tissues around the optic nerves (33%). Although about 30% of MOG-Ab patients had brain MRI lesions, they had fewer periventricular lesions than the 26 patients with relapsing-remitting multiple sclerosis (MS); none of these lesions were ovoid or perpendicular to the ventricle. Moreover, MOG-Ab patients did not meet the diagnostic criteria for definite neuromyelitis optica (NMO) and had less spinal cord involvement than the AQP4-Ab group. Four patients (23.5%) had poor visual outcomes (<0.2) or paraplegia.
MOG-Abs may be a disease-specific biomarker in adult patients with IDD who have a disease distinct from NMO or MS. The radiologic as well as clinical manifestations of MOG-Ab patients can be useful in their differential diagnosis.
评估髓鞘少突胶质细胞糖蛋白抗体(MOG-Ab)在中枢神经系统炎症性脱髓鞘疾病(IDD)患者中的临床相关性。
对 270 例成人 IDD 患者和 72 例对照者进行了基于活细胞的 MOG-Ab(IgG1 亚类)和抗水通道蛋白-4(AQP4-Ab)抗体检测。患者首先根据抗体阳性结果分为 MOG-Ab 或 AQP4-Ab 阳性,其余患者根据已发表的诊断标准分组。
17 例 IDD 患者(6.3%)存在 MOG-Ab,49 例患者(18.1%)存在 AQP4-Ab,无两者均有的患者。MOG-Ab 患者主要表现为孤立性视神经炎症状(83%)。其中三分之一的患者视神经炎复发,仅累及视神经,且所有患者均在疾病发病后 1 年内复发。在发病时,MOG-Ab 组的 MRI 独特地显示了神经周围增强,延伸到视神经周围的软组织(33%)。尽管约 30%的 MOG-Ab 患者有脑 MRI 病变,但它们比 26 例复发缓解型多发性硬化症(MS)患者的脑室周围病变少;这些病变均不是卵圆形或垂直于脑室。此外,MOG-Ab 患者不符合视神经脊髓炎(NMO)的明确诊断标准,脊髓受累程度也低于 AQP4-Ab 组。4 例(23.5%)患者视力预后不良(<0.2)或截瘫。
MOG-Ab 可能是成人 IDD 患者的一种疾病特异性生物标志物,其疾病与 NMO 或 MS 不同。MOG-Ab 患者的影像学和临床表现有助于鉴别诊断。