0
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Epidemiology of neuromyelitis optica spectrum disorder in Denmark (1998–2008, 2007–2014)

      research-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Epidemiological studies of the uncommon disorder neuromyelitis optica spectrum disorder (NMOSD) may be difficult to interpret because of the evolving nature of diagnostic criteria, differences in the definition and accuracy of NMOSD diagnosis, the completeness of case ascertainment, and variability in assays for the disease‐specific biomarker aquaporin‐4 (AQP4)‐IgG. A sub‐group of patients with the clinical syndrome NMOSD lack detectable AQP4‐IgG and in these cases an accurate diagnosis requires precise diagnostic algorithms and longitudinal follow‐up. Consecutive sets of criteria for NMO/NMOSD have been introduced during the two last decades. Such criteria need validation in different populations. Detection of other autoantibodies, such as IgG specific for myelin oligodendrocyte glycoprotein or for glial fibrillary acidic protein in a sub‐group of AQP4‐IgG–negative NMOSD patients, has improved over the past decade and may lead to overlap of the clinical syndromes/phenotypes. This review begins by summarizing current knowledge on the widening clinical spectrum of NMOSD. Subsequently, we describe two epidemiological studies from Denmark carried out in two different decades (1998–2008 and 2007–2014) and comment on the differences in study design, patient ascertainment, and interpretation of results. These factors may explain some of the observed differences, reflecting the complexity and providing a clear example of this development.

          Related collections

          Most cited references36

          • Record: found
          • Abstract: found
          • Article: found
          Is Open Access

          MOG encephalomyelitis: international recommendations on diagnosis and antibody testing

          Over the past few years, new-generation cell-based assays have demonstrated a robust association of autoantibodies to full-length human myelin oligodendrocyte glycoprotein (MOG-IgG) with (mostly recurrent) optic neuritis, myelitis and brainstem encephalitis, as well as with acute disseminated encephalomyelitis (ADEM)-like presentations. Most experts now consider MOG-IgG-associated encephalomyelitis (MOG-EM) a disease entity in its own right, immunopathogenetically distinct from both classic multiple sclerosis (MS) and aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorders (NMOSD). Owing to a substantial overlap in clinicoradiological presentation, MOG-EM was often unwittingly misdiagnosed as MS in the past. Accordingly, increasing numbers of patients with suspected or established MS are currently being tested for MOG-IgG. However, screening of large unselected cohorts for rare biomarkers can significantly reduce the positive predictive value of a test. To lessen the hazard of overdiagnosing MOG-EM, which may lead to inappropriate treatment, more selective criteria for MOG-IgG testing are urgently needed. In this paper, we propose indications for MOG-IgG testing based on expert consensus. In addition, we give a list of conditions atypical for MOG-EM (“red flags”) that should prompt physicians to challenge a positive MOG-IgG test result. Finally, we provide recommendations regarding assay methodology, specimen sampling and data interpretation.
            Bookmark
            • Record: found
            • Abstract: not found
            • Article: not found

            Glial fibrillary acidic protein immunoglobulin G as biomarker of autoimmune astrocytopathy: Analysis of 102 patients

              Bookmark
              • Record: found
              • Abstract: found
              • Article: found
              Is Open Access

              Complement activating antibodies to myelin oligodendrocyte glycoprotein in neuromyelitis optica and related disorders

              Background Serum autoantibodies against the water channel aquaporin-4 (AQP4) are important diagnostic biomarkers and pathogenic factors for neuromyelitis optica (NMO). However, AQP4-IgG are absent in 5-40% of all NMO patients and the target of the autoimmune response in these patients is unknown. Since recent studies indicate that autoimmune responses to myelin oligodendrocyte glycoprotein (MOG) can induce an NMO-like disease in experimental animal models, we speculate that MOG might be an autoantigen in AQP4-IgG seronegative NMO. Although high-titer autoantibodies to human native MOG were mainly detected in a subgroup of pediatric acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) patients, their role in NMO and High-risk NMO (HR-NMO; recurrent optic neuritis-rON or longitudinally extensive transverse myelitis-LETM) remains unresolved. Results We analyzed patients with definite NMO (n = 45), HR-NMO (n = 53), ADEM (n = 33), clinically isolated syndromes presenting with myelitis or optic neuritis (CIS, n = 32), MS (n = 71) and controls (n = 101; 24 other neurological diseases-OND, 27 systemic lupus erythematosus-SLE and 50 healthy subjects) for serum IgG to MOG and AQP4. Furthermore, we investigated whether these antibodies can mediate complement dependent cytotoxicity (CDC). AQP4-IgG was found in patients with NMO (n = 43, 96%), HR-NMO (n = 32, 60%) and in one CIS patient (3%), but was absent in ADEM, MS and controls. High-titer MOG-IgG was found in patients with ADEM (n = 14, 42%), NMO (n = 3, 7%), HR-NMO (n = 7, 13%, 5 rON and 2 LETM), CIS (n = 2, 6%), MS (n = 2, 3%) and controls (n = 3, 3%, two SLE and one OND). Two of the three MOG-IgG positive NMO patients and all seven MOG-IgG positive HR-NMO patients were negative for AQP4-IgG. Thus, MOG-IgG were found in both AQP4-IgG seronegative NMO patients and seven of 21 (33%) AQP4-IgG negative HR-NMO patients. Antibodies to MOG and AQP4 were predominantly of the IgG1 subtype, and were able to mediate CDC at high-titer levels. Conclusions We could show for the first time that a subset of AQP4-IgG seronegative patients with NMO and HR-NMO exhibit a MOG-IgG mediated immune response, whereas MOG is not a target antigen in cases with an AQP4-directed humoral immune response.
                Bookmark

                Author and article information

                Contributors
                nasgari@health.sdu.dk
                Journal
                Brain Behav
                Brain Behav
                10.1002/(ISSN)2157-9032
                BRB3
                Brain and Behavior
                John Wiley and Sons Inc. (Hoboken )
                2162-3279
                11 June 2019
                July 2019
                : 9
                : 7 ( doiID: 10.1002/brb3.2019.9.issue-7 )
                : e01338
                Affiliations
                [ 1 ] Department of Regional Health Research Odense Denmark
                [ 2 ] Department of Molecular Medicine University of Southern Denmark Odense Denmark
                [ 3 ] Department of Clinical Immunology Odense University Hospital Odense Denmark
                [ 4 ] Department of Radiology Aleris‐Hamlet Hospital Copenhagen Denmark
                [ 5 ] OPEN (Odense Patient data Explorative Network) Odense University Hospital Odense Denmark
                [ 6 ] Department of Clinical Research University of Southern Denmark Odense Denmark
                Author notes
                [*] [* ] Correspondence

                Nasrin Asgari, Department of Regional Health Research and Department of Molecular Medicine, University of Southern Denmark, J.B. Winsloewsvej 25, 2, DK‐5000 Odense, Denmark.

                Email: nasgari@ 123456health.sdu.dk

                Author information
                https://orcid.org/0000-0001-8551-0947
                Article
                BRB31338
                10.1002/brb3.1338
                6625475
                31187587
                02619c31-934e-4355-b3d8-ae27c2c88a33
                © 2019 The Authors. Brain and Behavior published by Wiley Periodicals, Inc.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                : 09 March 2019
                : 06 May 2019
                : 11 May 2019
                Page count
                Figures: 0, Tables: 0, Pages: 8, Words: 6857
                Categories
                Original Research
                Original Research
                Custom metadata
                2.0
                brb31338
                July 2019
                Converter:WILEY_ML3GV2_TO_NLMPMC version:5.6.5 mode:remove_FC converted:12.07.2019

                Neurosciences
                epidemiology,neuromyelitis optica spectrum disease
                Neurosciences
                epidemiology, neuromyelitis optica spectrum disease

                Comments

                Comment on this article