MOG Antibody Status Is Associated With Distinct Clinical Profiles and Treatment Responses in Chronic Relapsing Inflammatory Optic Neuropathy
Year: 2026
Type: article
Study design: Observational Study
Abstract: ABSTRACT Background 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 six European tertiary centers fulfilling diagnostic criteria for CRION were included. All underwent cell-based autoantibody testing. Clinical outcomes (visual acuity, 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 ARR before treatment (median [IQR] 2 [1-3] vs. 1 [1-2], p = 0.023), and a trend toward shorter inter-relapse intervals. Additional distinguishing features included higher frequencies of antinuclear antibody positivity, elevated CSF interleukin-6, and extensive optic neuritis 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. Conclusion CRION represents a heterogeneous syndrome encompassing distinct subgroups. MOG+ patients demonstrate higher disease activity but respond favorably to tocilizumab. Serological testing is critical for treatment stratification and preventing relapses.
Authors: Madalina Graure, Nathalie Nierobisch, Anthony De Vere-Tyndall, Thivya Pakeerathan, Ilya Ayzenberg, Jonathan A. Gernert, Joachim Havla, Marius Ringelstein, Orhan Aktas, Daria Tkachenko, Martin Werner Hümmert, Corinna Trebst, Nuria Cerdá-Fuertes, Athina Papadopoulou, Katrin Giglhuber, Rebecca Wicklein, Achim Berthele, Michael Weller, Veronika Kana, Patrick Roth, Marina Herwerth
Institutions: University of Zurich, ETH Zurich, University Hospital Zurich, St. Josef-Hospital, Ruhr University Bochum, LMU Klinikum, Institut für Klinische Neuroimmunologie, Ludwig-Maximilians-Universität München, Düsseldorf University Hospital, Heinrich Heine University Düsseldorf, Medizinische Hochschule Hannover, Klinikum Region Hannover, KRH Klinikum Agnes Karll Laatzen, University of Basel, University Hospital of Basel, Technical University of Munich
Language: English
Subfield: Clinical Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,450
OpenAlex ID: W7223246514