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

Chang, Vincent T. W.; Chang, Hyuk

doi:10.1111/nan.12574

Cited by 36 publications

(24 citation statements)

References 172 publications

(235 reference statements)

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“…5,6 The limited evidence so far suggests that clinical presentation in MOGAD differs from multiple sclerosis but overlaps with aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorders (AQP4+ NMOSD). 7 Given the different clinical outcome and treatment strategy compared to AQP4+ NMOSD, accurate diagnosis and evaluation of brain alterations in MOGAD would facilitate optimal treatment decisions and prognosis prediction. 5 MRI studies have mostly focused on focal lesion distribution to characterize MOGAD.…”

Section: Introductionmentioning

confidence: 99%

Brain structural and functional alterations in MOG antibody disease

et al. 2020

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Background: The impact of myelin oligodendrocyte glycoprotein antibody disease (MOGAD) on brain structure and function is unknown. Objectives: The aim of this study was to study the multimodal brain MRI alterations in MOGAD and to investigate their clinical significance. Methods: A total of 17 MOGAD, 20 aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorders (AQP4 + NMOSD), and 28 healthy controls (HC) were prospectively recruited. Voxel-wise gray matter (GM) volume, fractional anisotropy (FA), mean diffusivity (MD), and degree centrality (DC) were compared between groups. Clinical associations and differential diagnosis were determined using partial correlation and stepwise logistic regression. Results: In comparison with HC, MOGAD had GM atrophy in frontal and temporal lobe, insula, thalamus, and hippocampus, and WM fiber disruption in optic radiation and anterior/posterior corona radiata; DC decreased in cerebellum and increased in temporal lobe. Compared to AQP4 + NMOSD, MOGAD presented lower GM volume in postcentral gyrus and decreased DC in cerebellum. Hippocampus/parahippocampus atrophy associated with Expanded Disability Status Scale ( R = −0.55, p = 0.04) and California Verbal Learning Test ( R = 0.62, p = 0.031). The differentiation of MOGAD from AQP4 + NMOSD achieved an accuracy of 95% using FA in splenium of corpus callosum and DC in occipital gyrus. Conclusion: Distinct structural and functional alterations were identified in MOGAD. Hippocampus/parahippocampus atrophy associated with clinical disability and cognitive impairment.

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Section: Introductionmentioning

confidence: 99%

Brain structural and functional alterations in MOG antibody disease

et al. 2020

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“…Inflammatory damage is characterized by astrocyte loss and deposition of both immunoglobulins and complement, followed by neutrophil, monocyte, phagocyte and eosinophil infiltration [113]. Importantly, AQP4 distribution coincides with deposition patterns of IgG, IgM, and products of complement activation present in active NMO tissue [114,115], and MRI lesions of patients with NMO overlap with sites of high AQP4 expression [116]. AQP4-IgG is believed to determine internalization of the glutamate transporter EAAT2, limiting glutamate uptake from the extracellular space into astrocytes, also resulting in oligodendrocyte damage and myelin loss [117].…”

Section: Neuromyelitis Optica Spectrum-disordermentioning

confidence: 96%

Spinal Cord Involvement in MS and Other Demyelinating Diseases

2020

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Diagnostic accuracy is poor in demyelinating myelopathies, and therefore a challenge for neurologists in daily practice, mainly because of the multiple underlying pathophysiologic mechanisms involved in each subtype. A systematic diagnostic approach combining data from the clinical setting and presentation with magnetic resonance imaging (MRI) lesion patterns, cerebrospinal fluid (CSF) findings, and autoantibody markers can help to better distinguish between subtypes. In this review, we describe spinal cord involvement, and summarize clinical findings, MRI and diagnostic characteristics, as well as treatment options and prognostic implications in different demyelinating disorders including: multiple sclerosis (MS), neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, anti-myelin oligodendrocyte glycoprotein antibody-associated disease, and glial fibrillary acidic protein IgG-associated disease. Thorough understanding of individual case etiology is crucial, not only to provide valuable prognostic information on whether the disorder is likely to relapse, but also to make therapeutic decision-making easier and reduce treatment failures which may lead to new relapses and long-term disability. Identifying patients with monophasic disease who may only require acute management, symptomatic treatment, and subsequent rehabilitation, rather than immunosuppression, is also important.

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“…Along with complement, AQP4-IgG1 binds to AQP4 water channels (abundantly expressed on astrocyte end-feet processes) and exacerbates astrocyte injury. Source: [200]. associated with other autoimmune disorders such as systemic lupus erythematosus (SLE) and Graves' disease [19].…”

Section: Genetic Predispositionmentioning

confidence: 99%

Neuromyelitis optica spectrum disorders

Paul

Mondal

Bhattacharyya

et al. 2021

Journal of the Neurological Sciences

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The disease concept of Neuromyelitis Optica Spectrum Disorders(NMOSD) has undergone a significant change over the last two decades including the detection of Myelin Oligodendrocyte Glycoprotein(MOG) antibody in patients who are seronegative for aquaporin-4 antibody. Aquaporin-4 antibody positive NMOSD is now regarded as an immune astrocytopathy. Conversely, MOG antibody associated disease is known to target myelin rather than astrocytes, leading to an NMOSD syndrome with distinct clinical and radiological features. Incorporation of clinical features like area postrema syndrome, brainstem syndrome, diencephalic syndrome and cortical manifestations as core clinical characteristics into the revised diagnostic criteria has widened the clinical spectrum of NMOSD. With the development of these criteria, it is possible to make the diagnosis at an earlier stage so that effective immunosuppression can be instituted promptly for a better long-term prognosis. Newer therapeutic agents have been introduced for aquaporin-4 seropositive NMOSD disease; however, challenges remain in treating seronegative disease because of limited treatment options.

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

Cited by 36 publications

References 172 publications

Brain structural and functional alterations in MOG antibody disease

Brain structural and functional alterations in MOG antibody disease

Spinal Cord Involvement in MS and Other Demyelinating Diseases

Neuromyelitis optica spectrum disorders

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