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Int. J. Mol. Sci. 2016, 17(3), 273; doi:10.3390/ijms17030273

The Immunology of Neuromyelitis Optica—Current Knowledge, Clinical Implications, Controversies and Future Perspectives

1
Department of Neurology, Poznan University of Medical Sciences, 49 Przybyszewskiego St., 60-355 Poznan, Poland
2
Department of Neurochemistry and Neuropathology, Poznan University of Medical Sciences, 49 Przybyszewskiego St., 60-355 Poznan, Poland
3
Neuroimmunological Unit, Mossakowski Medical Research Centre, Polish Academy of Sciences, 5 Pawinskiego St., 02-106 Warsaw, Poland
*
Author to whom correspondence should be addressed.
Academic Editor: Christoph Kleinschnitz
Received: 12 January 2016 / Revised: 31 January 2016 / Accepted: 16 February 2016 / Published: 2 March 2016
(This article belongs to the Special Issue Advances in Multiple Sclerosis 2016)
View Full-Text   |   Download PDF [1941 KB, uploaded 2 March 2016]   |  

Abstract

Neuromyelitis optica (NMO) is an autoimmune, demyelinating disorder of the central nervous system (CNS) with typical clinical manifestations of optic neuritis and acute transverse myelitis attacks. Previously believed to be a variant of multiple sclerosis (MS), it is now considered an independent disorder which needs to be differentiated from MS. The discovery of autoantibodies against aquaporin-4 (AQP4-IgGs) changed our understanding of NMO immunopathogenesis and revolutionized the diagnostic process. AQP4-IgG is currently regarded as a specific biomarker of NMO and NMO spectrum disorders (NMOsd) and a key factor in its pathogenesis. Nevertheless, AQP4-IgG seronegativity in 10%–25% of NMO patients suggests that there are several other factors involved in NMO immunopathogenesis, i.e., autoantibodies against aquaporin-1 (AQP1-Abs) and antibodies against myelin oligodendrocyte glycoprotein (MOG-IgGs). This manuscript reviews current knowledge about NMO immunopathogenesis, pointing out the controversial issues and showing potential directions for future research. Further efforts should be made to broaden our knowledge of NMO immunology which could have important implications for clinical practice, including the use of potential novel biomarkers to facilitate an early and accurate diagnosis, and modern treatment strategies improving long-term outcome of NMO patients. View Full-Text
Keywords: neuromyelitis optica (NMO); neuromyelitis optica spectrum disorder (NMOsd); immunopathogenesis; neuroimmunology; aquaporin-4 immunoglobulin G (AQP4-IgG); aquaporin-1 antibody (AQP1-Ab); myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) neuromyelitis optica (NMO); neuromyelitis optica spectrum disorder (NMOsd); immunopathogenesis; neuroimmunology; aquaporin-4 immunoglobulin G (AQP4-IgG); aquaporin-1 antibody (AQP1-Ab); myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG)
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Jasiak-Zatonska, M.; Kalinowska-Lyszczarz, A.; Michalak, S.; Kozubski, W. The Immunology of Neuromyelitis Optica—Current Knowledge, Clinical Implications, Controversies and Future Perspectives. Int. J. Mol. Sci. 2016, 17, 273.

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