PT  - JOURNAL ARTICLE
AU  - Rinaldi, Simon
AU  - Davies, Alexander
AU  - Fehmi, Janev
AU  - Beadnall, Heidi N.
AU  - Wang, Justine
AU  - Hardy, Todd A.
AU  - Barnett, Michael H.
AU  - Broadley, Simon A.
AU  - Waters, Patrick
AU  - Reddel, Stephen W.
AU  - Irani, Sarosh R.
AU  - Brilot, Fabienne
AU  - Dale, Russell C.
AU  - Ramanathan, Sudarshini
AU  - ,
TI  - Overlapping central and peripheral nervous system syndromes in MOG antibody–associated disorders
AID  - 10.1212/NXI.0000000000000924
DP  - 2021 Jan 01
TA  - Neurology - Neuroimmunology Neuroinflammation
PG  - e924
VI  - 8
IP  - 1
4099  - http://nn.neurology.org/content/8/1/e924.short
4100  - http://nn.neurology.org/content/8/1/e924.full
SO  - Neurol Neuroimmunol Neuroinflamm2021 Jan 01; 8
AB  - Objective Antibodies to myelin oligodendrocyte glycoprotein (MOG) are associated with 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 immunologic characterization on those with likely PNS involvement.Results We identified 19 adults with MOGAD and PNS involvement without prior TM. All patients had CNS involvement including ON (bilateral [n = 3], unilateral [n = 3], and recurrent [n = 7]), a cortical lesion (n = 1), meningoencephalitis (n = 1), and subsequent TM (n = 4). Clinical phenotyping and neurophysiology were 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 IV immunoglobulin n = 9, rituximab n = 2, and plasmapheresis n = 1). We identified serum antibodies targeting neurofascin 155, contactin-associated protein 2, or GM1 in 4/16 patients with MOGAD PNS compared with 0/30 controls (p = 0.01). There was no binding to novel cell surface antigens using an in vitro myelinating sensory neuronal coculture 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 coexistent autoantibodies.AIDP=acute inflammatory demyelinating polyneuropathy; AQP4=aquaporin 4; BON=bilateral optic neuritis; CNTN1=contactin 1; CASPR1=contactin-associated protein-like 1; CASPR2=contactin-associated protein-like 2; CCPD=combined central and peripheral demyelination; CIDP=chronic inflammatory demyelinating polyneuropathy; HEK293=human embryonic kidney 293; IgG=immunoglobulin G; iPSC=induced pluripotent stem cell; IVIg=IV immunoglobulin; LETM=longitudinally extensive transverse myelitis; LGI1=leucine-rich glioma inactivated 1; MMN=multifocal motor neuropathy; MOG=myelin oligodendrocyte glycoprotein; MOGAD=MOG antibody–associated disorder; NCS=nerve conduction study; NF155=neurofascin 155; NF186=neurofascin 186; OCB=oligoclonal band; ON=optic neuritis; PMN=polymorphonuclear cell; PNS=peripheral nervous system; SFN=single fiber neuropathy; SSEP=somatosensory evoked potential; TM=transverse myelitis; UON=unilateral optic neuritis