PT  - JOURNAL ARTICLE
AU  - Papp, Viktoria
AU  - Magyari, Melinda
AU  - Aktas, Orhan
AU  - Berger, Thomas
AU  - Broadley, Simon A.
AU  - Cabre, Philippe
AU  - Jacob, Anu
AU  - Kira, Jun-ichi
AU  - Leite, Maria Isabel
AU  - Marignier, Romain
AU  - Miyamoto, Katsuichi
AU  - Palace, Jacqueline
AU  - Saiz, Albert
AU  - Sepulveda, Maria
AU  - Sveinsson, Olafur
AU  - Illes, Zsolt
TI  - Worldwide Incidence and Prevalence of Neuromyelitis Optica
AID  - 10.1212/WNL.0000000000011153
DP  - 2021 Jan 12
TA  - Neurology
PG  - 59--77
VI  - 96
IP  - 2
4099  - http://n.neurology.org/content/96/2/59.short
4100  - http://n.neurology.org/content/96/2/59.full
SO  - Neurology2021 Jan 12; 96
AB  - Objective Since the last epidemiologic review of neuromyelitis optica/neuromyelitis optica spectrum disorder (NMO/NMOSD), 22 additional studies have been conducted. We systematically review the worldwide prevalence, incidence, and basic demographic characteristics of NMOSD and provide a critical overview of studies.Methods PubMed, Ovid MEDLINE, and Embase using Medical Subject Headings and keyword search terms and reference lists of retrieved articles were searched from 1999 until August 2019. We collected data on the country; region; methods of case assessment and aquaporin-4 antibody (AQP4-Ab) test; study period; limitations; incidence (per 100,000 person-years); prevalence (per 100,000 persons); and age-, sex-, and ethnic group–specific incidence or prevalence.Results We identified 33 relevant articles. The results indicated the highest estimates of incidence and prevalence of NMOSD in Afro-Caribbean region (0.73/100 000 person-years [95% CI: 0.45–1.01] and 10/100 000 persons [95% CI: 6.8–13.2]). The lowest incidence and prevalence of NMOSD were found in Australia and New Zealand (0.037/100 000 person-years [95% CI: 0.036–0.038] and 0.7/100,000 persons [95% CI: 0.66–0.74]). There was prominent female predominance in adults and the AQP4-Ab–seropositive subpopulation. The incidence and prevalence peaked in middle-aged adults. African ethnicity had the highest incidence and prevalence of NMOSD, whereas White ethnicity had the lowest. No remarkable trend of incidence was described over time.Conclusion NMOSD is a rare disease worldwide. Variations in prevalence and incidence have been described among different geographic areas and ethnicities. These are only partially explained by different study methods and NMO/NMOSD definitions, highlighting the need for specifically designed epidemiologic studies to identify genetic effects and etiologic factors.AQP4-Ab=aquaporin-4 antibody; CBA=cell-based assay; IPND=International Panel for NMO Diagnosis; NMO=neuromyelitis optica; NMOSD=neuromyelitis optica spectrum disorder; MOG=myelin oligodendrocyte glycoprotein; ON=optic neuritis; TM=transverse myelitis