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

John J Chen¹, Eoin P Flanagan², Jiraporn Jitprapaikulsan³, Alfonso Sebastian S López-Chiriboga⁴, James P Fryer⁵, Jacqueline A Leavitt⁶, Brian G Weinshenker⁷, Andrew McKeon², Jan-Mendelt Tillema⁴, Vanda A Lennon⁸, W Oliver Tobin⁷, B Mark Keegan⁷, Claudia F Lucchinetti⁷, Orhun H Kantarci⁷, Collin M McClelland⁹, Michael S Lee⁹, Jeffrey L Bennett¹⁰, Victoria S Pelak¹⁰, Yanjun Chen¹¹, Gregory VanStavern¹², Ore-Ofe O Adesina¹³, Eric R Eggenberger¹⁴, Marie D Acierno¹⁵, Dean M Wingerchuk¹⁶, Paul W Brazis¹⁴, Jessica Sagen¹⁷, Sean J Pittock²

Affiliations

¹ Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: Chen.john@mayo.edu.
² Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA; Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.
³ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
⁴ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
⁵ Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
⁶ Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.
⁷ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.
⁸ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA; Department of Immunology, Mayo Clinic, Rochester, Minnesota, USA; Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.
⁹ Department of Ophthalmology and Visual Neurosciences, University of Minnesota, Minneapolis, Minnesota, USA.
¹⁰ Departments of Neurology and Ophthalmology, University of Colorado Denver School of Medicine, Aurora, Colorado, USA.
¹¹ Department of Ophthalmology, Neurology and Neurosurgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA.
¹² Departments of Ophthalmology and Visual Sciences and Neurology, Washington University, St. Louis School of Medicine, St. Louis, Missouri, USA.
¹³ Departments of Ophthalmology and Visual Science and Neurology, McGovern Medical School, Houston, Texas, USA.
¹⁴ Departments of Neurology and Neuro-Ophthalmology Mayo Clinic, Jacksonville, Florida, USA.
¹⁵ Department of Ophthalmology, Mayo Clinic, Scottsdale, Arizona, USA.
¹⁶ Department of Neurology, Mayo Clinic, Scottsdale, Arizona, USA.
¹⁷ Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.

PMID: 30055153
PMCID: PMC6371779
DOI: 10.1016/j.ajo.2018.07.020

Multicenter Study

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

John J Chen et al. Am J Ophthalmol. 2018 Nov.

. 2018 Nov:195:8-15.

doi: 10.1016/j.ajo.2018.07.020. Epub 2018 Jul 26.

Authors

Affiliations

¹ Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA. Electronic address: Chen.john@mayo.edu.
² Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA; Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.
³ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
⁴ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
⁵ Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
⁶ Department of Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.
⁷ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.
⁸ Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA; Department of Immunology, Mayo Clinic, Rochester, Minnesota, USA; Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.
⁹ Department of Ophthalmology and Visual Neurosciences, University of Minnesota, Minneapolis, Minnesota, USA.
¹⁰ Departments of Neurology and Ophthalmology, University of Colorado Denver School of Medicine, Aurora, Colorado, USA.
¹¹ Department of Ophthalmology, Neurology and Neurosurgery, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin, USA.
¹² Departments of Ophthalmology and Visual Sciences and Neurology, Washington University, St. Louis School of Medicine, St. Louis, Missouri, USA.
¹³ Departments of Ophthalmology and Visual Science and Neurology, McGovern Medical School, Houston, Texas, USA.
¹⁴ Departments of Neurology and Neuro-Ophthalmology Mayo Clinic, Jacksonville, Florida, USA.
¹⁵ Department of Ophthalmology, Mayo Clinic, Scottsdale, Arizona, USA.
¹⁶ Department of Neurology, Mayo Clinic, Scottsdale, Arizona, USA.
¹⁷ Center for MS and Autoimmune Neurology, Mayo Clinic, Rochester, Minnesota, USA.

PMID: 30055153
PMCID: PMC6371779
DOI: 10.1016/j.ajo.2018.07.020

Abstract

Purpose: To characterize the clinical phenotype of myelin oligodendrocyte glycoprotein antibody (MOG-IgG) optic neuritis.

Design: Observational case series.

Methods: 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.

Results: 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.

Conclusions: 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.

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Figures

**FIGURE 1.**
Kaplan-Meier estimates of time to (Top) Second optic neuritis attack and (Bottom) permanent severe visual loss.

**FIGURE 2.**
Fundus photograph and magnetic resonance imaging (MRI) findings of a patient with myelin oligodendrocyte glycoprotein antibody–positive optic neuritis. (Top) Funduscopy: Moderate bilateral optic disc edema with some peripapillary hemorrhages in the left eye. (Bottom) MRI with contrast: Bilateral prominent enhancement of optic nerve and sheath (perineural enhancement), extending along almost the entire nerve (red arrows).

See this image and copyright information in PMC

References

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

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

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