Global Prevalence of Neuromyelitis Optica Spectrum Disorder: A Systematic Review and Meta-Analysis with New Data from Rio de Janeiro, Brazil
Abstract
Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune astrocytopathy with geographic and ancestral variability in prevalence. Despite growing international epidemiological evidence, population-based prevalence studies of NMOSD remain scarce in Brazil.
Objective: The aim of the study was to integrate global evidence from a systematic review and meta-analysis with new regional prevalence data from southeastern Brazil using complementary epidemiological and statistical methods.
Methods: A systematic review was conducted to identify studies reporting crude NMOSD prevalence between 2000 and 2025. In parallel, a multi-source prevalence study was performed in the Volta Redonda-Barra Mansa intermediate region of Rio de Janeiro State. Crude prevalence, classical two-source capture-recapture estimators, and Bayesian modeling were applied. A random-effects meta-analysis combined international estimates with Brazilian data and compared pooled results across continents.
Results: We identified 44 eligible studies from 28 countries, with substantial heterogeneity reflecting differences in diagnostic criteria, case-ascertainment strategies, and geographic coverage. Crude NMOSD prevalence ranged from 0.23 to 10.0 per 100,000 inhabitants. In the Brazilian regional study, 24 confirmed cases among 782,004 inhabitants yielded a crude prevalence of 3.07 per 100,000, with capture-recapture estimates ranging from 3.53 to 5.60 per 100,000 and Bayesian posterior mean…
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