title: MOG-antibody associated demyelinating disease of the CNS: A clinical and pathological study in Chinese Han patients.
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.
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Lei
authors.initials: L
authors.lastname: Zhou
authors.affiliation: Department of Ophthalmology, Eye and ENT Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Yongheng
authors.initials: Y
authors.lastname: Huang
authors.affiliation: Department of Radiology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Haiqing
authors.initials: H
authors.lastname: Li
authors.affiliation: Department of Pathology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Jie
authors.initials: J
authors.lastname: Fan
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Jingzi
authors.initials: J
authors.lastname: Zhangbao
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Hai
authors.initials: H
authors.lastname: Yu
authors.affiliation: Department of Radiology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Yuxin
authors.initials: Y
authors.lastname: Li
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Jiahong
authors.initials: J
authors.lastname: Lu
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China; Department of Neurology, Jing'an District Centre Hospital of Shanghai, China.
authors.forename: Chongbo
authors.initials: C
authors.lastname: Zhao
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China.
authors.forename: Chuanzhen
authors.initials: C
authors.lastname: Lu
authors.affiliation: Department of Ophthalmology, Eye and ENT Hospital, Shanghai Medical College, Fudan University, China. Electronic address: wangmin83@yahoo.com.
authors.forename: Min
authors.initials: M
authors.lastname: Wang
authors.affiliation: Department of Neurology, Huashan Hospital, Shanghai Medical College, Fudan University, China. Electronic address: chao_quan@fudan.edu.cn.
authors.forename: Chao
authors.initials: C
authors.lastname: Quan
mesh_terms: D000328:Adult; D000368:Aged; D000818:Animals; D051401:Aquaporin 4 / Q000276:immunology*; D044466:Asian People / Q000208:ethnology; D001323:Autoantibodies / Q000097:blood*; D001921:Brain / Q000000981:diagnostic imaging; D002648:Child; D020278:Demyelinating Autoimmune Diseases, CNS / Q000097:blood / Q000276:immunology* / Q000473:pathology* / Q000503:physiopathology; D004797:Enzyme-Linked Immunosorbent Assay; D005123:Eye / Q000473:pathology; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000276:immunology*; D012160:Retina / Q000473:pathology; D013116:Spinal Cord / Q000000981:diagnostic imaging; D018709:Statistics, Nonparametric; D014792:Visual Acuity; D055815:Young Adult
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D063308:Myelin-Oligodendrocyte Glycoprotein