Abstract
We characterized a unique group of patients with neuromyelitis optica spectrum disorder (NMOSD) who carried autoantibodies of aquaporin-4 (AQP4) and myelin-oligodendrocyte glycoprotein (MOG). Among the 125 NMOSD patients, 10 (8.0%) were AQP4- and MOG-ab double positive, and 14 (11.2%) were MOG-ab single positive. The double-positive patients had a multiphase disease course with a high annual relapse rate (P=0.0431), and severe residual disability (P<0.0001). Of the double-positive patients, 70% had MS-like brain lesions, more severe edematous, multifocal regions on spinal magnetic resonance imaging (MRI), pronounced decreases of retinal nerve fiber layer thickness and atrophy of optic nerves. In contrast, patients with only MOG-ab had a higher ratio of monophasic disease course and mild residual disability. Spinal cord MRI illustrated multifocal cord lesions with mild edema, and brain MRIs showed more lesions around lateral ventricles. NMOSD patients carrying both autoantibodies to AQP4 and MOG existed and exhibited combined features of prototypic NMO and relapsing-remitting form of MS, whereas NMOSD with antibodies to MOG only exhibited an “intermediate” phenotype between NMOSD and MS. Our study suggests that antibodies against MOG might be pathogenic in NMOSD patients and that determination of anti-MOG antibodies maybe instructive for management of NMOSD patients.
Highlights
Damage to astrocytes mediated by aquaporin-4 antibody (AQP4-ab) has been implicated as the cause of neuromyelitis optica spectrum disorder (NMOSD) (Lennon et al, 2005; Ratelade et al, 2012; Saadoun et al, 2010)
The serum of 12/125 NMOSD patients were collected on no treatment (Table 1)
Sera from 10 of 125 NMOSD patients tested positive for both AQP4 and myelin-oligodendrocyte glycoprotein (MOG) autoantibodies
Summary
Damage to astrocytes mediated by aquaporin-4 antibody (AQP4-ab) has been implicated as the cause of neuromyelitis optica spectrum disorder (NMOSD) (Lennon et al, 2005; Ratelade et al, 2012; Saadoun et al, 2010). Despite using the best available assays, 10%–30% of patients with NMO and NMOSD still test negative for AQP4-ab (Granieri et al, 2012; Papadopoulos and Verkman, 2012; Waters et al, 2012). This suggests that other autoantigens might exist in AQP4-ab negative patients and drive the disease progress. Do not distinguish AQP4-ab-positive from -negative NMOSD patients. Published literature has suggested that these two groups of patients may have distinctive clinical features.
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