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New Advances in the Pathogenesis, Diagnosis and Treatment of NMOSD
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New Advances in the Pathogenesis, Diagnosis and Treatment of NMOSD

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: closed (31 January 2022) | Viewed by 21262

Special Issue Editor

Prof. Dr. Martin S. Weber

E-Mail Website
Guest Editor
Institute of Neuropathology, Department of Neurology, University Medical Center, 37099 Göttingen, Germany
Interests: multiple sclerosis; B lymphocytes; therapy; models; NMOSD; target development; immune system

Special Issue Information

Dear Colleagues,

The discovery of anti-aquaporin (AQP)-4 antibodies (abs) allowed us to progressively define neuromyelitis optica (NMO) as a disease entity separate from multiple sclerosis (MS). In our current understanding, AQP-4+ NMO is a classical autoimmune disease in which a peripheral ab responds against AQP-4 targets and continuously destroys astrocytes. Within the wider range of NMO-spectrum disorders (NMO-SD) a subgroup of AQP-4- patients produces abs against myelin oligodendrocyte glycoprotein (MOG). Accumulating evidence suggests that these patients may represent a disease entity by itself termed MOG-IgG-associated CNS demyelinating disorder.

In the upcoming Special Issue entitled “New Advances in the Pathogenesis, Diagnosis and Treatment of NMOSD”, we intend to represent all available radiological, immunological and histopathological data on patients with AQP-4+ NMO as well as MOG ab+ CNS demyelinating disease. Delineating and subsequently specifically treating these patients may be a significant step towards personalizing medicine in CNS demyelinating disorders.

Prof. Dr. Martin S. Weber
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Published Papers (4 papers)

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Review

28 pages, 397 KiB  
Review
Treatment of Neuromyelitis Optica Spectrum Disorders
by Koon-Ho Chan and Chi-Yan Lee
Int. J. Mol. Sci. 2021, 22(16), 8638; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22168638 - 11 Aug 2021
Cited by 24 | Viewed by 7051
Abstract
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune central nervous system (CNS) inflammatory disorder that can lead to serious disability and mortality. Females are predominantly affected, including those within the reproductive age. Most patients develop relapsing attacks of optic neuritis; longitudinally extensive transverse [...] Read more.
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune central nervous system (CNS) inflammatory disorder that can lead to serious disability and mortality. Females are predominantly affected, including those within the reproductive age. Most patients develop relapsing attacks of optic neuritis; longitudinally extensive transverse myelitis; and encephalitis, especially brainstem encephalitis. The majority of NMOSD patients are seropositive for IgG autoantibodies against the water channel protein aquaporin-4 (AQP4-IgG), reflecting underlying aquaporin-4 autoimmunity. Histological findings of the affected CNS tissues of patients from in-vitro and in-vivo studies support that AQP4-IgG is directly pathogenic in NMOSD. It is believed that the binding of AQP4-IgG to CNS aquaporin-4 (abundantly expressed at the endfoot processes of astrocytes) triggers astrocytopathy and neuroinflammation, resulting in acute attacks. These attacks of neuroinflammation can lead to pathologies, including aquaporin-4 loss, astrocytic activation, injury and loss, glutamate excitotoxicity, microglial activation, neuroinflammation, demyelination, and neuronal injury, via both complement-dependent and complement-independent pathophysiological mechanisms. With the increased understanding of these mechanisms underlying this serious autoimmune astrocytopathy, effective treatments for both active attacks and long-term immunosuppression to prevent relapses in NMOSD are increasingly available based on the evidence from retrospective observational data and prospective clinical trials. Knowledge on the indications and potential side effects of these medications are essential for a clear evaluation of the potential benefits and risks to NMOSD patients in a personalized manner. Special issues such as pregnancy and the coexistence of other autoimmune diseases require additional concern and meticulous care. Future directions include the identification of clinically useful biomarkers for the prediction of relapse and monitoring of the therapeutic response, as well as the development of effective medications with minimal side effects, especially opportunistic infections complicated by long-term immunosuppression. Full article
(This article belongs to the Special Issue New Advances in the Pathogenesis, Diagnosis and Treatment of NMOSD)
13 pages, 323 KiB  
Review
Neuromyelitis Optica Spectrum Disorder Treatment—Current and Future Prospects
by Marta Waliszewska-Prosół, Justyna Chojdak-Łukasiewicz, Sławomir Budrewicz and Anna Pokryszko-Dragan
Int. J. Mol. Sci. 2021, 22(6), 2801; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms22062801 - 10 Mar 2021
Cited by 11 | Viewed by 2855
Abstract
Neuromyelitis optica (NMO) is an immune-mediated demyelinative disorder of the central nervous system affecting mainly the optical nerves and the spinal cord. The recurrent course of the disease, with exacerbations and incomplete remissions, causes accumulating disability, which has a profound impact upon patients’ [...] Read more.
Neuromyelitis optica (NMO) is an immune-mediated demyelinative disorder of the central nervous system affecting mainly the optical nerves and the spinal cord. The recurrent course of the disease, with exacerbations and incomplete remissions, causes accumulating disability, which has a profound impact upon patients’ quality of life. The discovery of antibodies against aquaporin 4 (AQP4) and their leading role in NMO etiology and the formulation of diagnostic criteria have improved appropriate recognition of the disease. In recent years, there has been rapid progress in understanding the background of NMO, leading to an increasing range of treatment options. On the basis of a review of the relevant literature, the authors present currently available therapeutic strategies for NMO as well as ongoing research in this field, with reference to key points of immune-mediated processes involved in the background of the disease. Full article
(This article belongs to the Special Issue New Advances in the Pathogenesis, Diagnosis and Treatment of NMOSD)
17 pages, 1788 KiB  
Review
Hematopoietic Stem Cell Transplantation in Neuromyelitis Optica-Spectrum Disorders (NMO-SD): State-of-the-Art and Future Perspectives
by Giulia Ceglie, Laura Papetti, Massimiliano Valeriani and Pietro Merli
Int. J. Mol. Sci. 2020, 21(15), 5304; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms21155304 - 26 Jul 2020
Cited by 21 | Viewed by 4155
Abstract
Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD) are a group of autoimmune inflammatory disorders of the central nervous system (CNS). Understanding of the molecular basis of these diseases in the last decades has led to an important improvement in the treatment [...] Read more.
Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD) are a group of autoimmune inflammatory disorders of the central nervous system (CNS). Understanding of the molecular basis of these diseases in the last decades has led to an important improvement in the treatment of this disease, in particular, to the use of immunotherapeutic approaches, such as monoclonal antibodies and Hematopoietic Stem Cell Transplantation (HSCT). The aim of this review is to summarize the pathogenesis, biological basis and new treatment options of these disorders, with a particular focus on HSCT applications. Different HSCT strategies are being explored in NMOSD, both autologous and allogeneic HSCT, with the new emergence of therapeutic effects such as an induction of tolerance to auto-antigens and graft versus autoimmunity effects that can be exploited to hopefully treat a disease that still has prognosis. Full article
(This article belongs to the Special Issue New Advances in the Pathogenesis, Diagnosis and Treatment of NMOSD)
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30 pages, 1068 KiB  
Review
Differential Effects of MS Therapeutics on B Cells—Implications for Their Use and Failure in AQP4-Positive NMOSD Patients
by Jan Traub, Silke Häusser-Kinzel and Martin S. Weber
Int. J. Mol. Sci. 2020, 21(14), 5021; https://0-doi-org.brum.beds.ac.uk/10.3390/ijms21145021 - 16 Jul 2020
Cited by 19 | Viewed by 6269
Abstract
B cells are considered major contributors to multiple sclerosis (MS) pathophysiology. While lately approved disease-modifying drugs like ocrelizumab deplete B cells directly, most MS medications were not primarily designed to target B cells. Here, we review the current understanding how approved MS medications [...] Read more.
B cells are considered major contributors to multiple sclerosis (MS) pathophysiology. While lately approved disease-modifying drugs like ocrelizumab deplete B cells directly, most MS medications were not primarily designed to target B cells. Here, we review the current understanding how approved MS medications affect peripheral B lymphocytes in humans. These highly contrasting effects are of substantial importance when considering these drugs as therapy for neuromyelitis optica spectrum disorders (NMOSD), a frequent differential diagnosis to MS, which is considered being a primarily B cell- and antibody-driven diseases. Data indicates that MS medications, which deplete B cells or induce an anti-inflammatory phenotype of the remaining ones, were effective and safe in aquaporin-4 antibody positive NMOSD. In contrast, drugs such as natalizumab and interferon-β, which lead to activation and accumulation of B cells in the peripheral blood, lack efficacy or even induce catastrophic disease activity in NMOSD. Hence, we conclude that the differential effect of MS drugs on B cells is one potential parameter determining the therapeutic efficacy or failure in antibody-dependent diseases like seropositive NMOSD. Full article
(This article belongs to the Special Issue New Advances in the Pathogenesis, Diagnosis and Treatment of NMOSD)
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