Prevalence, incidence and clinical features of neuromyelitis optica spectrum disorders in northern Japan

Houzen, Hideki; Kano, Takahiro; Kondo, Kimito; Takahashi, Toshiyuki; Niino, Masaaki

doi:10.1136/jnnp-2022-330274

Cited by 4 publications

(1 citation statement)

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“…Almost two decades since the discovery of AQP4 antibodies, there is now accumulated data on the prevalence and incidence of AQP4+ NMOSD globally, with over 30 population-based studies published or presented from all continents except Africa (17,18). The data showed a racial preponderance where the prevalence is higher among East Asians (~5/100,000) and Black people (up to 10/100,000) when compared to White people (~1-1.5/100,000) and Austronesians (~1.5/100,000) (17)(18)(19)(20)(21). Despite being less well studied, there are now a number of population-based studies on the prevalence and incidence of MOGAD being reported, and it is time to review these data from around the world.…”

Section: Introductionmentioning

confidence: 99%

Epidemiology of myelin oligodendrocyte glycoprotein antibody-associated disease: a review of prevalence and incidence worldwide

Hor,

Fujihara

2023

Front. Neurol.

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Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is an inflammatory demyelinating disease of the central nervous system (CNS) with the presence of conformation-sensitive antibodies against MOG. The spectrum of MOGAD includes monophasic/relapsing optic neuritis, myelitis, neuromyelitis optica spectrum disorder (NMOSD) phenotype without aquaporin 4 (AQP4) antibodies, acute/multiphasic demyelinating encephalomyelitis (ADEM/MDEM)-like presentation, and brainstem and cerebral cortical encephalitis. There is no apparent female preponderance in MOGAD, and MOGAD can onset in all age groups (age at onset is approximately 30 years on average, and approximately 30% of cases are in the pediatric age group). While prevalence and incidence data have been available for AQP4+ NMOSD globally, such data are only beginning to accumulate for MOGAD. We reviewed the currently available data from population-based MOGAD studies conducted around the world: three studies in Europe, three in Asia, and one joint study in the Americas. The prevalence of MOGAD is approximately 1.3–2.5/100,000, and the annual incidence is approximately 3.4–4.8 per million. Among White people, the prevalence of MOGAD appears to be slightly higher than that of AQP4+ NMOSD. No obvious latitude gradient was observed in the Japanese nationwide survey. The data available so far showed no obvious racial preponderance or strong HLA associations in MOGAD. However, precedent infection was reported in approximately 20–40% of MOGAD cases, and this is worthy of further investigation. Co-existing autoimmune disorders are less common in MOGAD than in AQP4+ NMOSD, but NMDAR antibodies may occasionally be positive in patients with MOGAD. More population-based studies in different populations and regions are useful to further inform the epidemiology of this disease.

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