Myelin Oligodendrocyte Glycoprotein Antibody–Positive Optic Neuritis: Clinical Characteristics, Radiologic Clues, and Outcome

• Published in: American journal of ophthalmology Vol. 195; pp. 8 - 15
• Main Authors: Chen, John J., Flanagan, Eoin P., Jitprapaikulsan, Jiraporn, López-Chiriboga, Alfonso (Sebastian) S., Fryer, James P., Leavitt, Jacqueline A., Weinshenker, Brian G., McKeon, Andrew, Tillema, Jan-Mendelt, Lennon, Vanda A., Tobin, W. Oliver, Keegan, B. Mark, Lucchinetti, Claudia F., Kantarci, Orhun H., McClelland, Collin M., Lee, Michael S., Bennett, Jeffrey L., Pelak, Victoria S., Chen, Yanjun, VanStavern, Gregory, Adesina, Ore-Ofe O., Eggenberger, Eric R., Acierno, Marie D., Wingerchuk, Dean M., Brazis, Paul W., Sagen, Jessica, Pittock, Sean J.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.11.2018; Elsevier Limited
• Subjects: Adolescent; Adult; Age of Onset; Aged; Autoantibodies - blood; Charitable foundations; Child; Child, Preschool; Encephalomyelitis; Eye Pain - diagnosis; Female; Flow Cytometry; Fluorescent Antibody Technique, Indirect; Genotype & phenotype; Glycoproteins; HEK293 Cells; Humans; Immunoglobulin G - blood; Immunosuppressive Agents - therapeutic use; Inventors; Laboratories; Magnetic Resonance Imaging; Male; Middle Aged; Multiple sclerosis; Myelin-Oligodendrocyte Glycoprotein - immunology; NMR; Nuclear magnetic resonance; Ophthalmology; Optic nerve; Optic Neuritis - diagnostic imaging; Optic Neuritis - drug therapy; Optic Neuritis - immunology; Papilledema - diagnosis; Pharmaceuticals; Phenotype; Proteins; Retrospective Studies; Royalties; Transfection; Vision Disorders - physiopathology; Visual Acuity - physiology
• ISSN: 0002-9394; 1879-1891; 1879-1891
• DOI: 10.1016/j.ajo.2018.07.020

To characterize the clinical phenotype of myelin oligodendrocyte glycoprotein antibody (MOG-IgG) optic neuritis. Observational case series. Setting: 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 Measures: Clinical and radiologic characteristics and visual outcomes. Fifty-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. Manifestations 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.