Demographic and clinical features of neuromyelitis optica: A review
Open accessAbstract
The comparative clinical and demographic features of neuromyelitis optica (NMO) are not well known. In this review we analyzed peer-reviewed publications for incidence and prevalence, clinical phenotypes, and demographic features of NMO. Population-based studies from Europe, South East and Southern Asia, the Caribbean, and Cuba suggest that the incidence and prevalence of NMO ranges from 0.05–0.4 and 0.52–4.4 per 100,000, respectively. Mean age at onset (32.6–45.7) and median time to first relapse (8–12 months) was similar. Most studies reported an excess of disease in women and a relapsing course, particularly in anti-aquaporin 4 antibody (anti AQP4-IgG)-positive patients. Ethnicity may have a bearing on disease phenotype and clinical outcome. Despite limitations inherent to the review process, themes noted in clinical and demographic features of NMO among different populations promote a more global understanding of NMO and strategies to address it.
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NMOSD shows a strong gender bias (female:male ratio up to 9:1) and a mean age at onset of 39 years, although all age groups can be affected including children and the elderly.
“NMOSD shows a strong gender bias (female:male ratio up to 9:1) and a mean age at onset of 39 years 46 , although all age groups can be affected including children and the elderly.”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [46]
The Gender ratio section reports strong female predominance, and the Effect of age section documents both pediatric and late onset. However, “up to 9:1” understates the highest ratios reported, while the mean onset age of 39 years appears in Table 2 for a German cohort, not as an overall NMOSD mean; the review gives a range of means across case series. (LLM, conf=0.85)
Experimental — accuracy not yet measuredNMOSD is approximately 100 times less common than MS in European and North American populations but is relatively more common in Asia and Africa where MS is less common.
“NMOSD is approximately 100 times less common than MS in European and North American populations but is relatively more common in Asia and Africa where MS is less common.46”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [46]
The Prevalence section (page unavailable) reports that NMO prevalence varies across geographic and ethnic cohorts, and the Atlas of MS Database section gives figures for European countries, Japan, South Africa, and Southeast Asia. Neither section establishes that NMOSD is approximately 100 times less common than MS in Europe and North America, or that its frequency relative to MS is higher across Asia and Africa. (LLM, conf=0.77)
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Research works printed but not linked: 38
- [1]Wingerchuk DM Hogancamp WF O’Brien PC The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology 1999; 53: 1107– 1114. 10496275
- [2]Lennon V Wingerchuk D Kryzer T A serum autoantibody marker of neuromyelitis optica: Distinction from multiple sclerosis. Lancet 2004; 36445: 2106– 2112. 15589308
- [3]Wingerchuk DM Lennon VA Pittock SJ Revised diagnostic criteria for neuromyelitis optica. Neurology 2006; 66: 1485– 1489. 16717206
- [4]Wingerchuk DM Lennon VA Lucchinetti CF The spectrum of neuromyelitis optica. Lancet Neurol 2007; 6: 805– 815. 17706564
- [5]Pittock SJ Lennon VA Bakshi N Seroprevalence of aquaporin-4-IgG in a Northern California population representative cohort of multiple sclerosis. JAMA Neurol. 2014; 71: 1433– 1436. 25178362
- [6]Waters PJ McKeon A Leite MI Serologic diagnosis of NMO: A multicenter comparison of aquaporin-4-IgG assays. Neurology 2012; 78: 665– 671. 22302543 PMC3286228
- [7]Jarius S Wildemann B Aquaporin-4 antibodies (NMO-IgG) as a serological marker of neuromyelitis optica: A critical review of the literature. Brain Pathol 2013; 23: 661– 683. 24118483 PMC8028894
- [8]Asgari N Lillevang ST Skejoe HP A population-based study of neuromyelitis optica in Caucasians. Neurology 2011; 76: 1589– 1595. 21536639 PMC3269768
- [9]Cabre P Environmental changes and epidemiology of multiple sclerosis in the French West Indies. J Neurol Sci 2009; 286: 58– 61. 19520388
- [10]Cabrera-Gomez JA Kurtzke JF Gonzalez-Quevedo A An epidemiological study of neuromyelitis optica in Cuba. J Neurol 2009; 256: 35– 44. 19224310
- [11]Jacob A Panicker J Lythgoe D The epidemiology of neuromyelitis optica amongst adults in the Merseyside county of United Kingdom. J Neurol 2013; 260: 2134– 2137. 23689970
- [12]Aboul-Enein F Seifert-Held T Mader S Neuromyelitis optica in Austria in 2011: To bridge the gap between neuroepidemiological research and practice in a study population of 8.4 million people. PLoS One 2013; 8: e79649. 24223985 PMC3818238
- [13]Cossburn M Tackley G Baker K The prevalence of neuromyelitis optica in South East Wales. Eur J Neurol 2011; 19: 655– 659. 21967235
- [14]Houzen H Niino M Hirotani M Increased prevalence, incidence, and female predominance of multiple sclerosis in northern Japan. J Neurol Sci 2012; 323: 117– 122. 22995683
- [15]Pandit L Kundapur R Prevalence and patterns of demyelinating central nervous system disorders in urban Mangalore, South India. Mult Scler J 2014; 20: 1651– 1653. 24493471
- [16]Collongues N Marignier R Zéphir H Neuromyelitis optica in France: A multicenter study of 125 patients. Neurology 2010; 74: 736– 742. 20194912
- [17]Jarius S Ruprecht K Wildemann B, for the German Neuromyelitis Optica Study Group (NEMOS). Contrasting disease patterns in seropositive and seronegativeneuromyelitis optica: A multicentre study of 175 patients. J Neuroinflammation 2012; 9: 14. 22260418 PMC3283476
- [18]Höftberger R Sepúlveda M Armangue T Antibodies to MOG and AQP4 in adults with neuromyelitis optica and suspected limited forms of the disease. Mult Scler 2014; 10.1177/1352458514555785 [Epub ahead of print]. PMC4824843 25344373 · DOI
- [19]Mealy M Wingerchuck D Greenberg B Epidemiology of neuromyelitis optica in the United States, a multicenter analysis. Arch Neurol 2012; 69: 1176– 1180. 22733096
- [20]DB Bichuetti DB Oliveira EML Souza NA Neuromyelitis optica in Brazil: A study on clinical and prognostic factors. Mult Scler 2009; 15: 613– 619. 19299436
- [21]Pandit L Mustafa S Optimizing the management of neuromyelitis optica and spectrum disorders in resource poor settings: Experience from the Mangalore demyelinating disease registry. Ann Ind Academy Neurol 2013; 16: 572– 576. PMC3841603 24339582
- [22]Kim SH Kim W Li XF Clinical spectrum of CNS aquaporin-4 autoimmunity. Neurology 2012; 78: 1179– 1185. 22459676
- [23]Bizzoco E Lolli F Repice AM Prevalence of neuromyelitis optica spectrum disorder and phenotype distribution. J Neurol 2009; 256: 1891– 1898. 19479168
- [24]Siritho S Nakashima I Takahashi T AQP4 antibody-positive Thai cases: Clinical features and diagnostic problems. Neurology 2011; 77: 827– 834. 21813785 PMC3463102
- [25]Kitley J Leite MI Nakashima I Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan. Brain 2012; 135: 1834– 1849. 22577216
- [26]Collongues N Marignier R Zephir H Long-term follow-up of neuromyelitis optica with a pediatric onset. Neurology 2010; 75: 1084– 1088. 20855851
- [27]Quek AM McKeon A Lennon VA Effects of age and sex on aquaporin-4 autoimmunity. Arch Neurol 2012; 69: 1039– 1043. 22507888 PMC3746965
- [28]Collongues N Marignier R Jacob A Characterization of neuromyelitis optica and neuromyelitis optica spectrum disorder patients with a late onset. Mult Scler 2013; 20: 1086– 1094. 24323817
- [29]Kitley J Leite MI Küker W Longitudinally extensive transverse myelitis with and without aquaporin 4 antibodies. JAMA Neurol 2013; 70: 1375– 1378. 23999580
- [30]Marignier R Bernard-Valnet R Giraudon P Aquaporin-4 antibody-negative neuromyelitis optica: Distinct assay sensitivity-dependent entity. Neurology 2013; 80: 2194– 2200. 23658379
- [31]Downer JJ Leite MI Carter R Diagnosis of neuromyelitis optica (NMO) spectrum disorders: Is MRI obsolete? Neuroradiology 2012; 54: 279– 285. 21553012
- [32]Asgari N Skejoe HPB Lillevang ST Modifications of longitudinally extensive transverse myelitis and brainstem lesions in the course of neuromyelitis optica (NMO): A population-based, descriptive study. BMC Neurol 2013; 13: 33. 23566260 PMC3622587
- [33]Sepulveda M Blanco Y Rovira A Analysis of prognostic factors associated with longitudinally extensive transverse myelitis. Mult Scler 2013; 19: 742– 748. 23037550
- [34]Matsushita T Matsuoka T Isobe N Association of the HLA-DPB1*0501 allele with anti-aquaporin-4 antibody positivity in Japanese patients with idiopathic central nervous system demyelinating disorders. Tissue Antigens 2009; 73: 171– 176. 19140826
- [35]Brum DG Barreira AA dos Santos AC HLA-DRB association in neuromyelitis optica is different from that observed in multiple sclerosis. Mult Scler 2010; 16: 21– 29. 19995845
- [36]Deschamps R Paturel L Jeannin S Different HLA class II (DRB1 and DQB1) alleles determine either susceptibility or resistance to NMO and multiple sclerosis among the French Afro- Caribbean population. Mult Scler 2011; 17: 24– 31. 20861181
- [37]Zephir H Fajardy I Outteryck O Is neuromyelitis optica associated with human leukocyte antigen? Mult Scler 2009; 15: 571– 579. 19299434
- [38]Matiello M Kim HJ Kim W Familial neuromyelitis optica. Neurology 2010; 75: 310– 315. 20660861 PMC2918892
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Self-citationMIF -173G/C polymorphism is associated with NMO disease severity
Brill, L., Vaknin-Dembinsky, A., Zveik, O., Haham, N., Miller, K., & Benedek, G. (2020) · Journal of Neuroimmunology · doi:10.1016/j.jneuroim.2019.577120 · Cited by 5 · see citing works
- Authors
- Pandit, L., Asgari, N., Apiwattanakul, M., Palace, J., Paul, F., Leite, M., Kleiter, I., Chitnis, T., & the GJCF International Clinical Consortium & Biorepository for Neuromyelitis Optica
- Affiliations
- Nitte University (IN)
- University of Southern Denmark (DK)
- Neurological Institute of Thailand (TH)
- University of Oxford (GB)
- Charité – Universitätsmedizin Berlin (DE)
- St. Josef-Hospital (DE)
- Brigham and Women's Hospital (US)
- Corresponding author
- Pandit, L
- Venue
- Multiple Sclerosis Journal
- Publisher
- SAGE Publications
- Published
- 2015-04-28
- Volume
- 21
- Issue
- 7
- Pages
- 845-853
- ISSN
- 1352-4585 (ISSN-L)
- 1477-0970
- PMC
- PMC4463026
- OpenAlex
- W1977920771
- Cited by
- 341
- References
- 0
- Citations by year
- 2026: 10 · 2025: 27 · 2024: 24 · 2023: 27 · 2022: 29 · 2021: 47 · 2020: 45 · 2019: 38 · 2018: 37 · 2017: 28 · 2016: 22 · 2015: 7
- FWCI
- 4.59
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Locations
- Multiple Sclerosis Journal (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- MDC Repository (Max-Delbrueck-Center for Molecular Medicine) (acceptedVersion)
- eScholarship (California Digital Library) (acceptedVersion)
- University of Southern Denmark Research Portal (University of Southern Denmark) (acceptedVersion)
- eScholarship (California Digital Library) (submittedVersion, cc-by)
- Funders
- Guthy-Jackson Charitable Foundation
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
- Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
- Health Sciences › Medicine › Ophthalmology › Ocular Diseases and Behçet’s Syndrome
- Keywords
- neuromyelitis optica, incidence, prevalence, clinical phenotype, demographic factors, aquaporin-4 antibodies, relapse, age of onset, sex differences, ethnicity, disease outcome, population-based study
- MeSH terms
- Adult, Female, Humans, Male, Middle Aged, Neuromyelitis Optica
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