Clinico-epidemiological profile and outcome of pediatric neuromyelitis optica spectrum disorder at an eastern Indian tertiary care center

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a relapsing inflammatory central nervous system disorder. Patients either have antibodies to aquaporin 4 (AQ4)/myelin oligodendrocyte glycoprotein (MOG) or are double seronegatives (DSN). Aim: We aimed at comparing the clinico-epidemiological features and outcome of the subgroups of NMOSD. Design: Prospective longitudinal observational study. Materials and Methods: NMOSD was diagnosed by using the 2006 Wingerchuk criteria. Patients diagnosed between September 2016 and August 2018 were prospectively followed upto July 2020. Acute episodes were treated with steroids, and immunomodulators were started in patients with aquaporin 4 IgG (anti-AQ4+) and in relapsing cases of anti-MOG+ and DSN groups. Disability was assessed by using the Expanded Disability Status Scale (EDSS). Comparisons were made between patients who were anti-AQ4 and anti-MOG positive and those with DSN. Statistical analysis was done by SPSS 20.0 software. Results: Among 13 patients, the female:male ratio was 1.16:1, and the mean age of disease onset was 9.65 +/- 3.25 years. Overall, 15.38% patients were anti-AQ4+, 30.67% were anti-MOG+, 53.86% were DSN, 69.2% had relapsing disease, and 30.8% had monophasic disease (P = 0.11). The mean to relapse was 13.16 +/- 3.5 months; 61.5% patients had long segment myelitis and 53.86% had optic neuritis on their first presentation (P = 0.99). Cerebral syndrome occurred among one patient with anti-MOG+ and in three patients with DSN. The EDSS scores were significantly lower among patients who were anti-MOG+. Conclusion: The female:male ratio was more equitable and the age of disease onset was lower in our cohort compared with western data. There was no significant difference in the clinico-demographic characteristics among the three groups; however, outcome was better in the anti-MOG+ group. Rituximab was effective for recurrent relapses.