Worldwide prevalence of neuromyelitis optica spectrum disorders

Mori, Masahiro; Kuwabara, Satoshi; Paul, Friedemann

doi:10.1136/jnnp-2017-317566

Cited by 96 publications

(58 citation statements)

References 6 publications

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“…The prevalence of NMOSD is approximately 0.37–4.4 per 100 000 individuals with an increasing trend because of widespread AQP4‐IgG testing and improved AQP4‐IgG assay sensitivity . However, in contrast to MS, the prevalence of NMOSD is relatively similar, globally (rarely exceeding 5 per 100 000 individuals) . The incidence of NMOSD is higher in Martinique (10 per 100 000 individuals), West Indies, Asian and Afro‐American populations than in Caucasian populations .…”

Section: Neuromyelitis Optica Spectrum Disordermentioning

confidence: 52%

“…NMOSD is more prevalent in women than in men (9–10:1 in seropositive and 2:1 in seronegative patients) , and up to 90% of patients with relapsing disease are women . The prevalence of NMOSD is approximately 0.37–4.4 per 100 000 individuals with an increasing trend because of widespread AQP4‐IgG testing and improved AQP4‐IgG assay sensitivity . However, in contrast to MS, the prevalence of NMOSD is relatively similar, globally (rarely exceeding 5 per 100 000 individuals) .…”

Section: Neuromyelitis Optica Spectrum Disordermentioning

confidence: 99%

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Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorder

Chang

2019

Neuropathology Appl Neurobio

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Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorderNeuromyelitis optica is an autoimmune inflammatory disorder of the central nervous system that preferentially targets the spinal cord and optic nerve. Following the discovery of circulating antibodies against the astrocytic aquaporin 4 (AQP4) water channel protein, recent studies have expanded our knowledge of the unique complexities of the pathogenesis of neuromyelitis optica and its relationship with the immune response. This review describes and summarizes the recent advances in our understanding of the molecular mechanisms underlying neuromyelitis optica disease pathology and examines their potential as therapeutic targets. Additionally, we update the most recent research by proposing major unanswered questions regarding how peripheral AQP4 antibodies are produced and their entry into the central nervous system, the causes of AQP4-IgG-seronegative disease, why peripheral AQP4-expressing organs are spared from damage, and the impact of this disease on pregnancy.

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Section: Neuromyelitis Optica Spectrum Disordermentioning

confidence: 52%

Section: Neuromyelitis Optica Spectrum Disordermentioning

confidence: 99%

Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorder

Chang

2019

Neuropathology Appl Neurobio

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“…Given the rarity of NMOSD in Europe, an important strength of our study is the large sample size 40. Furthermore, we were able to distinguish disease-related changes from physiological changes by including a matched HC cohort.…”

Section: Discussionmentioning

confidence: 99%

Retinal ganglion cell loss in neuromyelitis optica: a longitudinal study

Oertel

Havla

Roca-Fernández

et al. 2018

J Neurol Neurosurg Psychiatry

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100

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This study clearly shows GCIP loss independent of ON attacks in aquaporin4-antibody-seropositive NMOSD. Potential explanations for progressive GCIP thinning include primary retinopathy, drug-induced neurodegeneration and retrograde neuroaxonal degeneration from lesions or optic neuropathy. pRNFL thickening in the patients presenting with attacks during F/U might be indicative of pRNFL susceptibility to inflammation.

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“…Autoantigens are considered to be unknown molecules on oligodendrocytes or myelin in the context of MS, and on aquaporin-4 (AQP4) water channels in astrocytes in the context of NMOSD. 9 The prevalence of NMO/ NMOSD ranges from 0.51 per 100 000 in Cuba to 4.4 per 100 000 in southern Denmark. 1,2,5,6 MS is globally the most prevalent chronic autoimmune inflammatory disease of the CNS.…”

Section: Introductionmentioning

confidence: 99%

“…7,8 The prevalence of MS has been reported as >100 per 100 000 and 0-20 per 100 000 in Europe/North American populations and in Asian populations, respectively. 9 The prevalence of NMO/ NMOSD ranges from 0.51 per 100 000 in Cuba to 4.4 per 100 000 in southern Denmark. 10 The classical clinical features of MS are as follows: (i) relapsing and remitting MS in the first years; that is, recurrent episodes of visible neurological dysfunction from which the individuals often recover; (ii) secondary progression leading to impaired mobility and cognition, typically 10-20 years after the first attack; and (iii) the primary slowly insidious progression of the disease course over time (primary progressive MS) in 10-15% of patients with MS. 6,[11][12][13][14] NMOSD is commonly characterized as having a relapsing-remitting clinical course, whereas a secondary progressive clinical course is uncommon.…”

Section: Introductionmentioning

confidence: 99%

Neuropathological features of “non‐motor” symptoms in multiple sclerosis and neuromyelitis optica

Kawachi

2019

Clinical & Exp Neuroim

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Multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are the two main autoimmune demyelinating disorders of the central nervous system. MS is defined as autoimmune oligodendrocytopathy (unknown autoantigens), and NMOSD is defined as autoimmune astrocytopathy or aquaporin-4 channelopathy. Both diseases can occur throughout the central nervous system, and do often cause various "motor" and "nonmotor" symptoms. "Non-motor" symptoms, including cognitive impairment, fatigue, pain and neuropsychological symptoms (e.g. depression, loneliness and anxiety), have gained renewed attention in MS and NMOSD since the past three decades, because they influence a patient's competence in daily life activities, including the participation in social activities and their employment status. Herein, we summarize the current concepts of the neuropathological processes of these "non-motor" symptoms in MS and NMOSD. These concepts help to define the novel disease-specific mechanisms of the two diseases, and to improve the quality of daily life of patients with MS and NMOSD.

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Worldwide prevalence of neuromyelitis optica spectrum disorders

Cited by 96 publications

References 6 publications

Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorder

Review: Recent advances in the understanding of the pathophysiology of neuromyelitis optica spectrum disorder

Retinal ganglion cell loss in neuromyelitis optica: a longitudinal study

Neuropathological features of “non‐motor” symptoms in multiple sclerosis and neuromyelitis optica

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