Overlapping central and peripheral nervous system syndromes in MOG antibody-associated disorders

Simon Rinaldi, Alexander Davies, Janev Fehmi, Heidi N Beadnall, Justine Wang, Todd A Hardy, Michael H Barnett, Simon A Broadley, Patrick Waters, Stephen W Reddel, Sarosh R Irani, Fabienne Brilot, Russell C Dale, Sudarshini Ramanathan, the Australian and New Zealand MOG Study Group

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Objective: Antibodies to myelin oligodendrocyte glycoprotein (MOG) are associated with central nervous system (CNS) demyelination inclusive of optic neuritis (ON) and transverse myelitis (TM). To examine whether peripheral nervous system (PNS) involvement is associated with MOG antibody-associated disorders (MOGAD), we performed detailed characterization of an Australasian MOGAD cohort. Methods: Using a live cell-based assay we diagnosed 271 adults with MOGAD (2013-2018), and performed detailed clinical and immunological characterization on those with likely PNS involvement. Results: We identified nineteen adults with MOGAD and PNS involvement without prior TM. All patients had CNS involvement including ON [bilateral (n=3), unilateral (n=3), recurrent (n=7)], a cortical lesion (n=1), meningoencephalitis (n=1), and subsequent TM (n=4). Clinical phenotyping and neurophysiology was consistent with acute inflammatory demyelinating polyneuropathy (n=1), myeloradiculitis (n=3), multifocal motor neuropathy (n=1), brachial neuritis (n=2), migrant sensory neuritis (n=3), and paresthesia and/or radicular limb pain (n=10). Onset MRI spine was consistent with myeloradiculitis with nerve root enhancement in 3/19, and normal in 16/19. Immunotherapy resulted in partial/complete PNS symptom resolution in 12/15 (80%) (steroids and/or intravenous immunoglobulin n=9, rituximab n=2, plasmapheresis n=1). We identified serum antibodies targeting neurofascin155, contactin associated protein 2, or GM1 in 4/16 MOGAD PNS patients compared to 0/30 controls (p=0.01). There was no binding to novel cell-surface antigens using an in vitro myelinating sensory neuronal co-culture model. Conclusions: Myeloradiculitis, combined central and peripheral demyelination syndromes, and inflammatory neuropathies may be associated with MOGAD, and may be immunotherapy responsive. We identified a subgroup who may have pathology mediated by co-existent autoantibodies.
Original languageEnglish
Number of pages32
JournalNeurology: Neuroimmunology & Neuroinflammation
Publication statusAccepted/In press - 16 Oct 2020

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