Comparison Of Clinical Characteristics, Therapeutic Responses And Outcomes In Myelin-Oligodendrocyte-Glycoprotein-Mog Associated Nmosd Versus Aqp4 Associated Nmosd In A Consecutive Cohort Of Sri Lankan Patients

Neuromyelitis optica spectrum disorder(NMOSD) has been reported in association with aquaporin-4(AQP4)-IgG positivity. Seronegative cases were later identified myelin-oligodendrocyte-glycoprotein(MOG)-IgG positivities. The clinical courses of these 2 groups differ. To compare the clinical phenotypes and outcomes of AQP4-IgG and MOG-IgG positive NMOSD patients. Sri Lankan patients fulfilling revised NMOSD diagnostic criteria in a CNS inflammatory demyelinating disorders(IDDs) cohort were included. Serostatus and clinical information were collected. Among 810 IDDs patients, 71 patients were identified, of whom 37 were AQP4-IgG positive and 34 were MOG-IgG1 positive. MOG-IgG1 positive were females 20/34(58.8%) while AQP4-IgG positives patients were 32/37(86.4%) (P = 0.009). AQP4-IgG positive NMOSD presented with isolated transverse myelitis(TM) in 12/34(32.4%), isolated optic neuritis(ON) in 8/34(21.6 %), and TM with ON in 8/34(21.6%) whereas MOG-IgG positive NMOSD presented with TM with ON in 9/37(26.4%), encephalitis with ON in 8/37(23.5%), and isolated ON in 6/37(17.6%). Area postrema syndrome (APS) was seen in the AQP4-IgG positive (8, 21.6%) compare with MOG-IgG positive patients (1, 2.9%) (P = 0.018). Eighty-seven percent of MOG-IgG1 positive and 97.2% of AQP4-IgG positive were treated during the first clinically encountered attack. Maintenance oral immunosuppressants were used in 47% and 94% in MOG-IgG1 positive and AQP4-IgG positive patients, respectively. Considering the mobility outcomes, 86% of MOG-IgG1 positive and 94% of AQP4-IgG positive NMOSD are currently walking unsupported. ON is more common in MOG-IgG positive patients than AQP4-IgG positive NMOSD whereas APS is rare in MOG-IgG NMOSD patients. Mobility outcomes of AQP4-IgG positive NMOSD are better than previous reports.