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MOG-antibody associated demyelinating disease of the CNS: A clinical and pathological study in Chinese Han patients

  • Author Footnotes
    1 These authors contributed equally to this manuscript.
    Lei Zhou
    Footnotes
    1 These authors contributed equally to this manuscript.
    Affiliations
    Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China
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  • Author Footnotes
    1 These authors contributed equally to this manuscript.
    Yongheng Huang
    Footnotes
    1 These authors contributed equally to this manuscript.
    Affiliations
    Department of Ophthalmology, Eye and ENT Hospital, Shanghai Medical College, Fudan University, China
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  • Author Footnotes
    1 These authors contributed equally to this manuscript.
    Haiqing Li
    Footnotes
    1 These authors contributed equally to this manuscript.
    Affiliations
    Department of Radiology, Huashan Hospital, Shanghai Medical College, Fudan University, China
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  • Author Footnotes
    1 These authors contributed equally to this manuscript.
    Jie Fan
    Footnotes
    1 These authors contributed equally to this manuscript.
    Affiliations
    Department of Pathology, Huashan Hospital, Shanghai Medical College, Fudan University, China
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  • Jingzi Zhangbao
    Affiliations
    Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China
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  • Hai Yu
    Affiliations
    Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China
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  • Yuxin Li
    Affiliations
    Department of Radiology, Huashan Hospital, Shanghai Medical College, Fudan University, China
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  • Jiahong Lu
    Affiliations
    Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China
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  • Chongbo Zhao
    Affiliations
    Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China

    Department of Neurology, Jing'an District Centre Hospital of Shanghai, China
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  • Chuanzhen Lu
    Affiliations
    Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China
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  • Min Wang
    Correspondence
    Correspondence to: M. Wang, Department of Ophthalmology, Eye and ENT Hospital of Fudan University, 83 Fenyang Road, Shanghai 200031, China.
    Affiliations
    Department of Ophthalmology, Eye and ENT Hospital, Shanghai Medical College, Fudan University, China
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  • Chao Quan
    Correspondence
    Correspondence to: C. Quan, Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, 12 Wulumuqi Zhong Road, Shanghai 200040, China.
    Affiliations
    Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China
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  • Author Footnotes
    1 These authors contributed equally to this manuscript.

      Highlights

      • Twelve of the 86 unselected patients with suspected IDDs are MOG-ab positive.
      • Ten of the 12 patients with MOG-ab have optic neuritis during the disease course.
      • Intracranial lesions are common and of heterogeneity in MOG-ab associated IDDs.
      • Large white matter lesions in the brain are observed in MOG-ab associated IDDs.
      • The pathological feature of MOG-ab associated brain lesion is distinct from NMO/SD.

      Abstract

      We aim to evaluate the clinical relevance of MOG-ab in a cohort of Chinese Han adults with CNS inflammatory demyelinating diseases (IDDs). MOG-ab and AQP4-ab were examined through a fixed cell based indirect immune-fluorescence assay in 86 patients with CNS-IDDs. MOG-ab was positive in 12 patients, while AQP4-ab was positive in 31 patients; none double positives. Optic neuritis (ON) was the most frequent symptom at onset (75.0%) or during the whole disease course (83.3%) of MOG-ab associated IDDs (MOG-IDDs); 79.5% of the episodes involved only the optic nerve in MOG-IDDs. MOG-ab related ON (MOG-ON) usually caused severe visual impairment, longitudinally extensive optic nerve lesion with anterior enhancement and perineural soft tissue enhancement, responded well to steroid, but still could leave remarkable thinning of retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC). MOG-IDDs had less spinal cord involvement compared to AQP4-ab mediated NMO/SD. Heterogeneous brain lesions existed in 66.7% of the patients with MOG-IDDs. Large, edematous white matter lesions were observed with the pathological feature of obvious demyelination yet preservation of astrocyte and axon, fundamentally different from the astrocytopathy typically seen in NMO/SD. Our investigations suggest that MOG-ab mediates a distinct disease entity separate from NMO/SD.
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