Abstract
Objective: We examined a cohort of adults with aquaporin-4 (AQP4) antibody–negative neuromyelitis optica/neuromyelitis optica spectrum disorder (NMO/NMOSD) for antibodies to myelin oligodendrocyte glycoprotein (MOG).
Methods: We performed a flow cytometry cell-based assay using live human lentivirus–transduced cells expressing full-length surface MOG. Serum was tested in 23 AQP4 antibody–negative NMO/NMOSD patients with bilateral and/or recurrent optic neuritis (BON, n = 11), longitudinally extensive transverse myelitis (LETM, n = 10), and sequential BON and LETM (n = 2), as well as in patients with multiple sclerosis (MS, n = 76) and controls (n = 52).
Results: MOG antibodies were detected in 9/23 AQP4 antibody–negative patients with NMO/NMOSD, compared to 1/76 patients with MS and 0/52 controls (p < 0.001). MOG antibodies were detected in 8/11 patients with BON, 0/10 patients with LETM, and 1/2 patients with sequential BON and LETM. Six of 9 MOG antibody–positive patients had a relapsing course. MOG antibody–positive patients had prominent optic disc swelling and were more likely to have a rapid response to steroid therapy and relapse on steroid cessation than MOG antibody–negative patients (p = 0.034 and p = 0.029, respectively). While 8/9 MOG antibody–positive patients had good follow-up visual acuity, one experienced sustained visual impairment, 3 had retinal nerve fiber layer thinning, and one had residual spinal disability.
Conclusions: MOG antibodies have a strong association with BON and may be a useful clinical biomarker. MOG antibody–associated BON is a relapsing disorder that is frequently steroid responsive and often steroid dependent. Failure to recognize the disorder early and institute immunotherapy promptly may be associated with sustained impairment.
Classification of evidence: This study provides Class II evidence that MOG antibodies are associated with AQP4 antibody–negative BON (sensitivity 69%, 95% confidence interval [CI] 42%–87%; specificity 99%, 95% CI 93.7%–99.8%).
GLOSSARY
- ADEM=
- acute disseminated encephalomyelitis;
- AQP4=
- aquaporin-4;
- BON=
- bilateral and/or recurrent optic neuritis;
- CI=
- confidence interval;
- CRION=
- chronic relapsing inflammatory optic neuropathy;
- EDSS=
- Expanded Disability Status Scale;
- FACS=
- fluorescence-activated cell sorting;
- HEK293=
- human embryonic kidney 293;
- Ig=
- immunoglobulin;
- LETM=
- longitudinally extensive transverse myelitis;
- MD=
- mean deviation;
- MFI=
- mean fluorescence intensity;
- MOG=
- myelin oligodendrocyte glycoprotein;
- mRS=
- modified Rankin Scale;
- MS=
- multiple sclerosis;
- NMO=
- neuromyelitis optica;
- NMOSD=
- neuromyelitis optica spectrum disorder;
- OCT=
- optical coherence tomography;
- ON=
- optic neuritis;
- RNFL=
- retinal nerve fiber layer;
- VFSS=
- visual functional system score
Footnotes
Go to Neurology.org/nn for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. The Article Processing Charge was paid by the authors.
Supplemental data at Neurology.org/nn
- Received June 1, 2014.
- Accepted in final form September 11, 2014.
- © 2014 American Academy of Neurology
This is an open access article distributed under the terms of the Creative Commons Attribution-Noncommercial No Derivative 3.0 License, which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially.
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