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

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 au...

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Main Authors: 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
Format: Article
Language:English
Published: Frontiers Media S.A. 2020-06-01
Series:Frontiers in Neurology
Subjects:
MOG
Online Access:https://www.frontiersin.org/article/10.3389/fneur.2020.00501/full
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language English
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author Jyh Yung Hor
Nasrin Asgari
Ichiro Nakashima
Simon A. Broadley
Simon A. Broadley
M. Isabel Leite
Najib Kissani
Anu Jacob
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
spellingShingle Jyh Yung Hor
Nasrin Asgari
Ichiro Nakashima
Simon A. Broadley
Simon A. Broadley
M. Isabel Leite
Najib Kissani
Anu Jacob
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
Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide
Frontiers in Neurology
neuromyelitis optica spectrum disorder
NMOSD
AQP4
MOG
prevalence
incidence
author_facet Jyh Yung Hor
Nasrin Asgari
Ichiro Nakashima
Simon A. Broadley
Simon A. Broadley
M. Isabel Leite
Najib Kissani
Anu Jacob
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
author_sort Jyh Yung Hor
title Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide
title_short Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide
title_full Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide
title_fullStr Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide
title_full_unstemmed Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence Worldwide
title_sort epidemiology of neuromyelitis optica spectrum disorder and its prevalence and incidence worldwide
publisher Frontiers Media S.A.
series Frontiers in Neurology
issn 1664-2295
publishDate 2020-06-01
description 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 <1/million population. Among East Asians, the prevalence is higher, at ~3.5/100,000 population, while the prevalence in Blacks may be up to 10/100,000 population. For MOG-antibody disease, hospital-based studies largely do not observe any significant racial preponderance so far. This disorder comprises a significant proportion of NMOSD cases that are AQP4-antibody-seronegative. A recent Dutch nationwide study reported the annual incidence of MOG-antibody disease as 1.6/million population (adult: 1.3/million, children: 3.1/million). Clinical and radiological differences between AQP4-antibody and MOG-antibody associated diseases have led to interest in the revisions of NMOSD definition and expanded stratification based on detection of a specific autoantibody biomarker. More population-based studies in different geographical regions and racial groups will be useful to further inform the prevalence and incidence of NMOSD and their antibody-specific subgroups. Accessibility to AQP4-antibody and MOG-antibody testing, which is limited in many centers, is a challenge to overcome. Environmental and genetic studies will be useful accompaniments to identify other potential pathogenetic factors and specific biomarkers in NMOSD.
topic neuromyelitis optica spectrum disorder
NMOSD
AQP4
MOG
prevalence
incidence
url https://www.frontiersin.org/article/10.3389/fneur.2020.00501/full
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spelling doaj-e6ed929680c240cc85feb6da407e466d2020-11-25T02:58:02ZengFrontiers Media S.A.Frontiers in Neurology1664-22952020-06-011110.3389/fneur.2020.00501543047Epidemiology of Neuromyelitis Optica Spectrum Disorder and Its Prevalence and Incidence WorldwideJyh Yung Hor0Nasrin Asgari1Ichiro Nakashima2Simon A. Broadley3Simon A. Broadley4M. Isabel Leite5Najib Kissani6Anu Jacob7Anu Jacob8Romain Marignier9Brian G. Weinshenker10Friedemann Paul11Sean J. Pittock12Jacqueline Palace13Dean M. Wingerchuk14Jacinta M. Behne15Michael R. Yeaman16Kazuo Fujihara17Department of Neurology, Penang General Hospital, Penang, MalaysiaDepartment of Neurology, Institute of Molecular Medicine, University of Southern Denmark, Odense, DenmarkDepartment of Neurology, Tohoku Medical and Pharmaceutical University, Sendai, JapanMenzies Health Institute Queensland, Griffith University, Southport, QLD, AustraliaDepartment of Neurology, Gold Coast University Hospital, Southport, QLD, AustraliaNuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United KingdomNeurology Department and Neuroscience Research Laboratory of Marrakech Medical School, University Hospital Mohammed VI, Marrakech, MoroccoWalton Centre NHS Foundation Trust, Liverpool, United KingdomCleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates0Service de Neurologie, Sclérose en Plaques, Pathologies de la Myéline et Neuro-inflammation, and Centre de Référence des Maladies Inflammatoires Rares du Cerveau et de la Moelle, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Lyon, France1Department of Neurology, Mayo Clinic, Rochester, MN, United States2NeuroCure Clinical Research Center, Charité—Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, and Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine and Charité—Universitätsmedizin Berlin, Berlin, Germany1Department of Neurology, Mayo Clinic, Rochester, MN, United StatesNuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom3Department of Neurology, Mayo Clinic, Scottsdale, AZ, United States4The Guthy-Jackson Charitable Foundation, Beverly Hills, CA, United States5Divisions of Molecular Medicine and Infectious Diseases, David Geffen School of Medicine at UCLA, Los Angeles and Harbor-UCLA Medical Center, Lundquist Institute for Biomedical Innovation at Harbor-UCLA Medical Center, Torrance, CA, United States6Department of Multiple Sclerosis Therapeutics, Fukushima Medical University School of Medicine, and Multiple Sclerosis and Neuromyelitis Optica Center, Southern TOHOKU Research Institute for Neuroscience, Koriyama, JapanNeuromyelitis 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 <1/million population. Among East Asians, the prevalence is higher, at ~3.5/100,000 population, while the prevalence in Blacks may be up to 10/100,000 population. For MOG-antibody disease, hospital-based studies largely do not observe any significant racial preponderance so far. This disorder comprises a significant proportion of NMOSD cases that are AQP4-antibody-seronegative. A recent Dutch nationwide study reported the annual incidence of MOG-antibody disease as 1.6/million population (adult: 1.3/million, children: 3.1/million). Clinical and radiological differences between AQP4-antibody and MOG-antibody associated diseases have led to interest in the revisions of NMOSD definition and expanded stratification based on detection of a specific autoantibody biomarker. More population-based studies in different geographical regions and racial groups will be useful to further inform the prevalence and incidence of NMOSD and their antibody-specific subgroups. Accessibility to AQP4-antibody and MOG-antibody testing, which is limited in many centers, is a challenge to overcome. Environmental and genetic studies will be useful accompaniments to identify other potential pathogenetic factors and specific biomarkers in NMOSD.https://www.frontiersin.org/article/10.3389/fneur.2020.00501/fullneuromyelitis optica spectrum disorderNMOSDAQP4MOGprevalenceincidence