• John J Chen, Eoin P Flanagan, Jiraporn Jitprapaikulsan, Alfonso Sebastian S López-Chiriboga, James P Fryer, Jacqueline A Leavitt, Brian G Weinshenker, Andrew McKeon, Jan-Mendelt Tillema, Vanda A Lennon, W Oliver Tobin, B Mark Keegan, Claudia F Lucchinetti, Orhun H Kantarci, Collin M McClelland, Michael S Lee, Jeffrey L Bennett, Victoria S Pelak, Yanjun Chen, Gregory VanStavern, Ore-Ofe O Adesina, Eric R Eggenberger, Marie D Acierno, Dean M Wingerchuk, Paul W Brazis, Jessica Sagen, and Sean J Pittock.
• Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: Chen.john@mayo.edu.
• Am. J. Ophthalmol. 2018 Nov 1; 195: 8-15.

PurposeTo characterize the clinical phenotype of myelin oligodendrocyte glycoprotein antibody (MOG-IgG) optic neuritis.DesignObservational case series.MethodsSetting: Multicenter. Patient/Study Population: Subjects meeting inclusion criteria: (1) history of optic neuritis; (2) seropositivity (MOG-IgG binding index > 2.5); 87 MOG-IgG-seropositive patients with optic neuritis were included (Mayo Clinic, 76; other medical centers, 11). MOG-IgG was detected using full-length MOG-transfected live HEK293 cells in a clinically validated flow cytometry assay.Main Outcome MeasuresClinical and radiologic characteristics and visual outcomes.ResultsFifty-seven percent were female and median age at onset was 31 (range 2-79) years. Median number of optic neuritis attacks was 3 (range 1-8), median follow-up 2.9 years (range 0.5-24 years), and annualized relapse rate 0.8. Average visual acuity (VA) at nadir of worst attack was count fingers. Average final VA was 20/30; for 5 patients (6%) it was ≤20/200 in either eye. Optic disc edema and pain each occurred in 86% of patients. Magnetic resonance imaging showed perineural enhancement in 50% and longitudinally extensive involvement in 80%. Twenty-six patients (30%) had recurrent optic neuritis without other neurologic symptoms, 10 (12%) had single optic neuritis, 14 (16%) had chronic relapsing inflammatory optic neuropathy, and 36 (41%) had optic neuritis with other neurologic symptoms (most neuromyelitis optica spectrum disorder-like phenotype or acute disseminated encephalomyelitis). Only 1 patient was diagnosed with MS (MOG-IgG-binding index 2.8; normal range ≤ 2.5). Persistent MOG-IgG seropositivity occurred in 61 of 62 (98%). A total of 61% received long-term immunosuppressant therapy.ConclusionsManifestations of MOG-IgG-positive optic neuritis are diverse. Despite recurrent attacks with severe vision loss, the majority of patients have significant recovery and retain functional vision long-term.Copyright © 2018 Elsevier Inc. All rights reserved.

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