MOG Antibody Status Is Associated With Distinct Clinical Profiles and Treatment Responses in Chronic Relapsing Inflammatory Optic Neuropathy
15 affiliations
- 1Department of Neurology, University Hospital Zurich, Switzerland
- 2Neuroscience Center Zurich, University of Zurich and ETH Zurich, Switzerland
- 3Department of Diagnostic and Interventional Neuroradiology, University Hospital Zurich, Switzerland
- 4Department of Neurology, St. Josef Hospital, Ruhr University Bochum, Germany
- 5Institute of Clinical Neuroimmunology, LMU University Hospital, Ludwig-Maximilians-University Munich, Germany
- 6Department of Neurology, Medical Faculty and University Hospital, Heinrich Heine University Düsseldorf, Germany
- 7Department of Neurology, Centre for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf, Germany
- 8Department of Neurology, Hannover Medical School, Germany
- 9PRACTIS Clinician Scientist Program, Dean's Office for Academic Career Development, Hannover Medical School, Germany
- 10Department of Neurology, Klinikum Agnes Karl Laatzen/ Klinikum Region Hannover, Germany
- 11Department of Neurology, University Hospital Basel, Switzerland
- 12Department of Clinical Research, University of Basel, Switzerland
- 13Translational Imaging in Neurology (ThINK) Basel, Department of Medicine and Biomedical Engineering, University Hospital Basel and University of Basel, Switzerland
- 14Department of Neurology, School of Medicine and Health, Technical University Munich, Germany ; and
- 15Institute of Pharmacology and Toxicology, University of Zurich, Switzerland .
Abstract
Background and Objectives: Chronic relapsing inflammatory optic neuropathy (CRION) is a steroid-dependent form of optic neuritis with incompletely understood pathophysiology. The identification of myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) in a substantial patient subset has challenged the diagnostic and therapeutic management. The aim of this study was to investigate clinical profiles and treatment outcomes of patients with CRION, comparing MOG-IgG-positive (MOG+) and seronegative (MOG−) subgroups.
Methods: Patients from 6 European tertiary centers fulfilling diagnostic criteria for CRION were included. All underwent cell-based autoantibody testing. Clinical outcomes (visual acuity and annualized relapse rate), laboratory and imaging findings (MRI, OCT), and treatment responses were retrospectively analyzed.
Results: Sixty patients were included (median age 33 years; 70% female); 27 (45%) were MOG+. MOG+ CRION was associated with later onset, higher annualized relapse rate (ARR) before treatment (median [IQR] 2 [1–3] vs 1 [1–2], p = 0.023), and a trend toward shorter interrelapse intervals. Additional distinguishing features included higher frequencies of antinuclear antibody positivity, elevated CSF interleukin-6, and extensive enhancement of the optic nerve on MRI. Relapse burden correlated with visual acuity decline and retinal thinning. In MOG+ patients, monoclonal antibody therapy reduced the ARR (n = 21; 2 [1–3] vs 0 [0–2], p = 0.024), primarily driven by tocilizumab (n = 11; 2 [1–3] vs 0 [0–1], p = 0.023). In MOG− patients, rituximab and azathioprine showed a trend toward ARR reduction.
Discussion: CRION represents a heterogeneous syndrome encompassing distinct subgroups. Although MOG+ patients demonstrate higher disease activity, tocilizumab might represent a therapeutic option, though further validation is needed. Serologic testing is critical for treatment stratification and preventing relapses.
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BibTeX
@article{graure2026antibody,
title = {{MOG Antibody Status Is Associated With Distinct Clinical Profiles and Treatment Responses in Chronic Relapsing Inflammatory Optic Neuropathy}},
author = {Madalina Graure and Nathalie Nierobisch and Anthony De Vere-Tyndall and Thivya Pakeerathan and Ilya Ayzenberg and Jonathan A. Gernert and Joachim Havla and Marius Ringelstein and Orhan Aktas and Daria Tkachenko and Martin Werner Hümmert and Corinna Trebst and Nuria Cerdá-Fuertes and Athina Papadopoulou and Katrin Giglhuber and Rebecca Wicklein and Achim Berthele and Michael Weller and Veronika Kana and Patrick Roth and Marina Herwerth},
journal = {Neurology® Neuroimmunology \& Neuroinflammation},
year = {2026},
month = oct,
doi = {10.1212/nxi.0000000000200668},
url = {https://doi.org/10.1212/nxi.0000000000200668},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - MOG Antibody Status Is Associated With Distinct Clinical Profiles and Treatment Responses in Chronic Relapsing Inflammatory Optic Neuropathy
AU - Madalina Graure
AU - Nathalie Nierobisch
AU - Anthony De Vere-Tyndall
AU - Thivya Pakeerathan
AU - Ilya Ayzenberg
AU - Jonathan A. Gernert
AU - Joachim Havla
AU - Marius Ringelstein
AU - Orhan Aktas
AU - Daria Tkachenko
AU - Martin Werner Hümmert
AU - Corinna Trebst
AU - Nuria Cerdá-Fuertes
AU - Athina Papadopoulou
AU - Katrin Giglhuber
AU - Rebecca Wicklein
AU - Achim Berthele
AU - Michael Weller
AU - Veronika Kana
AU - Patrick Roth
AU - Marina Herwerth
PY - 2026
DA - 2026/10/22
JO - Neurology® Neuroimmunology & Neuroinflammation
DO - 10.1212/nxi.0000000000200668
UR - https://doi.org/10.1212/nxi.0000000000200668
AB - Background and Objectives: Chronic relapsing inflammatory optic neuropathy (CRION) is a steroid-dependent form of optic neuritis with incompletely understood pathophysiology. The identification of myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) in a substantial patient subset has challenged the diagnostic and therapeutic management. The aim of this study was to investigate clinical profiles and treatment outcomes of patients with CRION, comparing MOG-IgG-positive (MOG+) and seronegative (MOG−) subgroups. Methods: Patients from 6 European tertiary centers fulfilling diagnostic criteria for CRION were included. All underwent cell-based autoantibody testing. Clinical outcomes (visual acuity and annualized relapse rate), laboratory and imaging findings (MRI, OCT), and treatment responses were retrospectively analyzed. Results: Sixty patients were included (median age 33 years; 70% female); 27 (45%) were MOG+. MOG+ CRION was associated with later onset, higher annualized relapse rate (ARR) before treatment (median [IQR] 2 [1–3] vs 1 [1–2], p = 0.023), and a trend toward shorter interrelapse intervals. Additional distinguishing features included higher frequencies of antinuclear antibody positivity, elevated CSF interleukin-6, and extensive enhancement of the optic nerve on MRI. Relapse burden correlated with visual acuity decline and retinal thinning. In MOG+ patients, monoclonal antibody therapy reduced the ARR (n = 21; 2 [1–3] vs 0 [0–2], p = 0.024), primarily driven by tocilizumab (n = 11; 2 [1–3] vs 0 [0–1], p = 0.023). In MOG− patients, rituximab and azathioprine showed a trend toward ARR reduction. Discussion: CRION represents a heterogeneous syndrome encompassing distinct subgroups. Although MOG+ patients demonstrate higher disease activity, tocilizumab might represent a therapeutic option, though further validation is needed. Serologic testing is critical for treatment stratification and preventing relapses.
SN - 2332-7812
ER -