University of Cincinnati College of Medicine, Department of Neurology and Rehabilitation Medicine, Waddell Center for Multiple Sclerosis, 260 Stetson Street, Suite 2300, Cincinnati, OH 45267-0525, United States.
University Hospitals Cleveland Medical Center, Case Western Reserve University School of Medicine, Neurological Institute, Bolwell, 5th floor, 11100 Euclid Avenue, Cleveland, OH 44106-1716, United States.
Mult Scler Relat Disord. 2021 Jul;52:102964. doi: 10.1016/j.msard.2021.102964. Epub 2021 Apr 20.
Background MOG-IgG-associated disease (MOGAD) in adults typically presents as a monophasic or relapsing optic, spinal, or opticospinal neuroinflammatory syndrome. Current recommendations discourage testing for MOG-IgG in patients with clinical or paraclinical findings more typical of MS, or in patients with a progressive clinical course. However, this approach may impede identification of the full phenotypic spectrum of this recently described disorder. Methods We retrospectively reviewed charts of 39 MOG-IgG-seropositive patients from two Ohio-based neuroimmunology centers to identify unusual disease patterns. Those with a progressive course were included in this case series. Results We describe five cases of progressive myelopathy associated with MOG-IgG. Most patients had features suggestive of MS, including typical MRI and cerebrospinal fluid findings. However, MOG-IgG positive patients with progressive myelopathy showed poor response to MS disease modifying therapy and better response to intravenous immunoglobulins similar to previous reports on MOGAD patients. Conclusion MOG-IgG-seropositive patients may present with progressive myelopathy and may have a clinical and radiologic phenotype suggestive of primary progressive or secondary progressive MS, or progressive solitary sclerosis. MOG-IgG testing should be considered in patients with progressive myelopathy, especially if clinically worsening on MS therapy.
成人 MOG-IgG 相关疾病（MOGAD）通常表现为单相或复发性视神经、脊髓或视神经脊髓神经炎症综合征。目前的建议不鼓励在临床或辅助检查结果更符合多发性硬化症（MS）的患者中，或在进行性临床病程的患者中检测 MOG-IgG。然而，这种方法可能会阻碍对这种新描述疾病的完整表型谱的识别。
我们回顾了来自俄亥俄州两个神经免疫中心的 39 名 MOG-IgG 阳性患者的图表，以确定不常见的疾病模式。那些进行性病程的患者被纳入本病例系列。
我们描述了五例与 MOG-IgG 相关的进行性脊髓病。大多数患者具有 MS 的特征性表现，包括典型的 MRI 和脑脊液发现。然而，进行性脊髓病的 MOG-IgG 阳性患者对 MS 疾病修正治疗反应不佳，对静脉注射免疫球蛋白反应较好，这与之前关于 MOGAD 患者的报告相似。
MOG-IgG 阳性患者可能表现为进行性脊髓病，且可能具有临床和影像学表型，提示原发性进行性或继发性进行性 MS，或进行性孤立性硬化症。在进行性脊髓病患者中，应考虑进行 MOG-IgG 检测，特别是在 MS 治疗后临床恶化的患者。
