Prevalence and incidence of neuromyelitis optica spectrum disorder, aquaporin-4 antibody-positive NMOSD and MOG antibody-positive disease in Oxfordshire, UK

O’Connell, Karen; Hamilton-Shield, Antonia; Woodhall, Mark; Messina, Silvia; Mariano, Romina; Waters, Patrick; Ramdas, Sithara; Leite, Maria Isabel; Palace, Jacqueline

doi:10.1136/jnnp-2020-323158

Cited by 67 publications

(46 citation statements)

References 12 publications

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“…In the past two decades, epidemiological data of NMOSD were accumulated and they provided important insights into this disease 7 . Interestingly, while the prevalence of MS was high in the predominantly Caucasian populations in Europe and North America, their NMOSD prevalence was relatively low, with population‐based studies reported a prevalence of ~ 1‐1.5/100 000 among Caucasians 8 . e2 On the contrary, prevalence of NMOSD was found to be higher among East Asians 9,10 and Blacks 8 .…”

Section: Epidemiology: Prevalence and Incidencementioning

confidence: 99%

Neuromyelitis optica spectrum disorder in Asia: Epidemiology and risk factors

Hor

Wong

et al. 2021

Neurology & Clinical Neurosc

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In Asia, prior to the discovery of aquaporin 4 (AQP4) antibody, many cases of neuromyelitis optica spectrum disorder (NMOSD) were misdiagnosed as multiple sclerosis (MS) or the so-called opticospinal multiple sclerosis (OSMS). The AQP4 antibody discovery was an important milestone in the field of NMOSD, leading to correct diagnosis and reclassification of NMOSD cases, and the subsequent appropriate therapy. This is especially pertinent in Asia where the prevalence of MS is generally much lower than the Caucasian populations. East Asians have a higher NMOSD prevalence rate (~4/100 000) than Caucasians (~1-1.5/100 000) and other Asian racial groups (Austronesians: 1.2-1.5/100 000; South Asians: ~1/100 000; Arabs: ~1-1.5/100 000). Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) comprises cases of AQP4-antibody-negative NMOSD, optic neuritis, myelitis, and certain unique phenotypes such as cases with acute disseminated encephalomyelitis (ADEM)-like presentation, and cerebral cortical encephalitis. MOGAD appears not too uncommon among Japanese, Chinese, Sri Lankans and Indians, but data in different Asian populations and geographical locations are needed. There exist some differences in clinical features of NMOSD and MOGAD between Asians and other populations. As for risk factors, genetic studies have identified certain HLA associations with NMOSD among Asians that were different from other populations. North-South latitudinal gradient as seen in MS was not observed in NMOSD in Japan and China. Seasonal variation and preceding infection are among some risk factors worth further investigations. Population-based MOGAD study in Asia, and further genetic and environmental studies are useful to further inform the epidemiology and potential pathogenetic mechanism of this unique neuroinflammatory disease. K E Y W O R D S epidemiology, myelin oligodendrocyte glycoprotein antibody-associated disease, neuromyelitis optica spectrum disorder, population study, risk factor | 275 HOR et al.

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Section: Epidemiology: Prevalence and Incidencementioning

confidence: 99%

Neuromyelitis optica spectrum disorder in Asia: Epidemiology and risk factors

Hor

Wong

et al. 2021

Neurology & Clinical Neurosc

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“…NMOSD is clinically characterized by attacks of uni-or bilateral optic neuritis (ON), acute myelitis and/or brain/brainstem encephalitis (8) and is associated with specific autoantibodies (aAbs) such as anti-aquaporin-4 (AQP4), targeting an astrocytic water channel (9), or anti-myelin oligodendrocyte glycoprotein (MOG)-IgG (5,10). Approximately 60% of all NMOSD patients are positive for anti-AQP4-IgG (11)(12)(13). Anti-AQP4-IgG is therefore thought to play a key or even causal role in disease pathogenesis, characterizing the anti-AQP4-IgG positive subset of NMOSD as a channelopathy (11).…”

Section: Introductionmentioning

confidence: 99%

Anti-aquaporin 4 IgG Is Not Associated With Any Clinical Disease Characteristics in Neuromyelitis Optica Spectrum Disorder

et al. 2021

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Background: Neuromyelitis optica spectrum disorder (NMOSD) is a clinically defined, inflammatory central nervous system (CNS) disease of unknown cause, associated with humoral autoimmune findings such as anti-aquaporin 4 (AQP4)-IgG. Recent clinical trials showed a benefit of anti-B cell and anti-complement-antibodies in NMOSD, suggesting relevance of anti-AQP4-IgG in disease pathogenesis.Objective: AQP4-IgG in NMOSD is clearly defined, yet up to 40% of the patients are negative for AQP4-IgG. This may indicate that AQP4-IgG is not disease-driving in NMOSD or defines a distinct patient endotype.Methods: We established a biobank of 63 clinically well-characterized NMOSD patients with an extensive annotation of 351 symptoms, patient characteristics, laboratory results and clinical scores. We used phylogenetic clustering, heatmaps, principal component and longitudinal causal interference analyses to test for the relevance of anti-AQP4-IgG.Results: Anti-AQP4-IgG was undetectable in 29 (46%) of the 63 NMOSD patients. Within anti-AQP4-IgG-positive patients, anti-AQP4-IgG titers did not correlate with clinical disease activity. Comparing anti-AQP4-IgG-positive vs. -negative patients did not delineate any clinically defined subgroup. However, anti-AQP4-IgG positive patients had a significantly (p = 0.022) higher rate of additional autoimmune diagnoses.Conclusion: Our results challenge the assumption that anti-AQP4-IgG alone plays a disease-driving role in NMOSD. Anti-AQP4-IgG might represent an epiphenomenon associated with NMOSD, may represent one of several immune mechanisms that collectively contribute to the pathogenesis of this disease or indeed, anti-AQP4-IgG might be the relevant factor in only a subgroup of patients.

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“…23 Several sociodemographic features also differ in MOGAD compared with AQP4-IgG positive NMOSD, including younger age of onset, a higher proportion of Caucasian ethnicity and less pronounced female predominance in MOGAD. 1,[23][24][25][26][27][28][29][30] These immunobiological and clinical distinctions have led many experts to consider MOGAD as a distinct clinical entity, separate from NMOSD. 27,[31][32][33][34][35] With the accumulation of data from several retrospective studies that describe the clinical spectrum of MOGAD, it is now evident that ON represents the most common clinical manifestation of the disease in adults.…”

Section: Introductionmentioning

confidence: 99%

“…In a nationwide epidemiological study from the Netherlands, the mean incidence of MOGAD between 2014 and 2017 was 0.16/100 000 persons/year, with higher incidence rates in children than in adults (0.31/100 000/year vs 0.13/100 000/year, respectively) 22 . In a recent epidemiological study from the UK, the prevalence and incidence rates of MOGAD were nearly twice as common as AQP4‐IgG‐positive NMOSD 23 …”

Section: Introductionmentioning

confidence: 99%

“…Several sociodemographic features also differ in MOGAD compared with AQP4‐IgG positive NMOSD, including younger age of onset, a higher proportion of Caucasian ethnicity and less pronounced female predominance in MOGAD 1,23‐30 . These immunobiological and clinical distinctions have led many experts to consider MOGAD as a distinct clinical entity, separate from NMOSD 27,31‐35 …”

Section: Introductionmentioning

confidence: 99%

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Practical recognition tools of immunoglobulin G serum antibodies against the myelin oligodendrocyte glycoprotein‐positive optic neuritis and its clinical implications

Lotan

Oertel

Chien

et al. 2021

Clinical & Exp Neuroim

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Myelin oligodendrocyte glycoprotein (MOG)‐associated disease is an autoimmune disease of the central nervous system, associated with the presence of immunoglobulin G serum antibodies against MOG. Recent data have allowed characterization of the clinical spectrum of MOG‐associated disease, which is now considered a new disease entity, distinct from multiple sclerosis and neuromyelitis optica spectrum disorders. Optic neuritis is the most common clinical presentation of MOG‐associated disease in adults, both at disease onset and during the disease course, and has several distinct clinical and paraclinical features. Immunoglobulin G serum antibodies against MOG‐positive optic neuritis is often bilateral and associated with optic disc swelling and a longitudinally extensive abnormal magnetic resonance imaging signal involving the retrobulbar portion of the optic nerve. The visual acuity during the acute attack is severely decreased, and the response to corticosteroids is often rapid and prominent. However, early relapses after steroid cessation are common, and a subset of patients is left with a permanent visual disability. In this review, we discuss the clinical and paraclinical features of immunoglobulin G serum antibodies against MOG‐positive optic neuritis in adults, and focus on the distinctive features that can enable its early diagnosis. Therapeutical considerations at the acute stage and for relapse prevention are further deliberated.

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Prevalence and incidence of neuromyelitis optica spectrum disorder, aquaporin-4 antibody-positive NMOSD and MOG antibody-positive disease in Oxfordshire, UK

Cited by 67 publications

References 12 publications

Neuromyelitis optica spectrum disorder in Asia: Epidemiology and risk factors

Neuromyelitis optica spectrum disorder in Asia: Epidemiology and risk factors

Anti-aquaporin 4 IgG Is Not Associated With Any Clinical Disease Characteristics in Neuromyelitis Optica Spectrum Disorder

Practical recognition tools of immunoglobulin G serum antibodies against the myelin oligodendrocyte glycoprotein‐positive optic neuritis and its clinical implications

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