Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide

Autor: Jyh Yung Hor, Nasrin Asgari, Ichiro Nakashima, Simon A. Broadley, M. Isabel Leite, Najib Kissani, Anu Jacob, Romain Marignier, Brian G. Weinshenker, Friedemann Paul, Sean J. Pittock, Jacqueline Palace, Dean M. Wingerchuk, Jacinta M. Behne, Michael R. Yeaman, Kazuo Fujihara
Jazyk: angličtina
Rok vydání: 2020
Předmět:
Zdroj: Frontiers in Neurology, Vol 11 (2020)
Druh dokumentu: article
ISSN: 1664-2295
DOI: 10.3389/fneur.2020.00501
Popis: Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon inflammatory disease of the central nervous system, manifesting clinically as optic neuritis, myelitis, and certain brain and brainstem syndromes. Cases clinically diagnosed as NMOSD may include aquaporin 4 (AQP4)-antibody-seropositive autoimmune astrocytopathic disease, myelin oligodendrocyte glycoprotein (MOG)-antibody-seropositive inflammatory demyelinating disease, and double-seronegative disease. AQP4-antibody disease has a high female-to-male ratio (up to 9:1), and its mean age at onset of ~40 years is later than that seen in multiple sclerosis. For MOG-antibody disease, its gender ratio is closer to 1:1, and it is more common in children than in adults. Its clinical phenotypes differ but overlap with those of AQP4-antibody disease and include acute disseminated encephalomyelitis, brainstem and cerebral cortical encephalitis, as well as optic neuritis and myelitis. Double-seronegative disease requires further research and clarification. Population-based studies over the past two decades report the prevalence and incidence of NMOSD in different populations worldwide. One relevant finding is the varying prevalence observed in different racial groups. Consistently, the prevalence of NMOSD among Whites is ~1/100,000 population, with an annual incidence of
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