RT Journal Article
SR Electronic
T1 Worldwide Incidence and Prevalence of Neuromyelitis Optica
JF Neurology
JO Neurology
FD Lippincott Williams & Wilkins
SP 59
OP 77
DO 10.1212/WNL.0000000000011153
VO 96
IS 2
A1 Papp, Viktoria
A1 Magyari, Melinda
A1 Aktas, Orhan
A1 Berger, Thomas
A1 Broadley, Simon A.
A1 Cabre, Philippe
A1 Jacob, Anu
A1 Kira, Jun-ichi
A1 Leite, Maria Isabel
A1 Marignier, Romain
A1 Miyamoto, Katsuichi
A1 Palace, Jacqueline
A1 Saiz, Albert
A1 Sepulveda, Maria
A1 Sveinsson, Olafur
A1 Illes, Zsolt
YR 2021
UL http://n.neurology.org/content/96/2/59.abstract
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