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Neuromyelitis Optica Spectrum Disorders – from pathophysiology to treatment
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  • Neuromyelitis Optica Spectrum Disorders – from pathophysiology to treatment
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Neuromyelitis Optica Spectrum Disorders – from pathophysiology to treatment

Authors

  • Jessica Adriana Kałuża University Clinical Centre of the Medical University of Warsaw, Banacha 1a, 02-097 Warsaw, Poland https://orcid.org/0009-0002-5050-2538
  • Oliwia Bochenek Grochowski Hospital, Grenadierów 51/59, 04-073 Warsaw, Poland https://orcid.org/0009-0005-1482-2544
  • Aleksandra Nowak Infant Jesus Clinical Hospital UCC MUW, Lindleya 4, 02-005 Warsaw, Poland https://orcid.org/0009-0009-6602-4017
  • Mateusz Koper National Medical Institute of the Ministry of the Interior and Administration, Wołoska 137, 02-507 Warsaw, Poland https://orcid.org/0000-0002-1048-2774
  • Adrian Konaszczuk Standalone Public Health Care Facility in Świdnik ul. Leśmiana 4, 21-040 Świdnik https://orcid.org/0009-0006-5775-5035
  • Klaudia Ratyna Infant Jesus Clinical Hospital UCC MUW, Lindleya 4, 02-005 Warsaw, Poland https://orcid.org/0009-0005-2235-1105
  • Oliwia Kozyra Prague hospital dedicated to the Transfiguration of the Lord Sp. z o. o., Aleja Solidarności 67, 03-401, Warsaw, Poland https://orcid.org/0009-0004-4882-3126
  • Zofia Szypuła Medical University of Warsaw, Żwirki i Wigury 61, 02-091 Warsaw, Poland https://orcid.org/0009-0007-1671-5587
  • Katarzyna Paluch Medical University of Warsaw, Żwirki i Wigury 61, 02-091 Warsaw, Poland https://orcid.org/0009-0004-2190-855X
  • Małgorzata Skarbek Provincial Hospital Center of the Jelenia Gora Valley, Oginskiego 6, 58-506 Jelenia Gora https://orcid.org/0000-0001-6227-4283

DOI:

https://doi.org/10.12775/QS.2024.21.54342

Keywords

Neuromyelitis Optica Spectrum Disorders, demyelinating disease, Aquaporin-4-antibody, immunosuppressive therapies, Acute treatment, Long-term management

Abstract

Neuromyelitis optica spectrum disorders (NMOSD) are a group of autoimmune diseases characterized by severe, inflammatory attacks predominantly affecting the optic nerves and spinal cord and central nervous system. NMOSD presents with clinical features such as optic neuritis, acute myelitis, and area postrema syndrome, making it a challenging condition to diagnose due to its varied clinical manifestations. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first attack. Historically considered a variant of multiple sclerosis, NMOSD is now recognized as a distinct entity, largely due to the discovery of the pathogenic role of aquaporin-4 (AQP4) antibodies. Approximately 75% of patients have antibodies against aquaporin-4, a water channel expressed on astrocytes. These antibodies target astrocytes, leading to complement activation, inflammation, and subsequent damage to the central nervous system. Relapses are treated with high-dose steroids and plasma exchanges to prevent residual disability. Advances in understanding the pathophysiology of NMOSD have paved the way for targeted therapies, including monoclonal antibodies that inhibit complement activation, B-cell depletion strategies, and interventions targeting interleukin-6 signaling. Four preventive immunotherapies have now been approved for AQP4-IgG-positive NMOSD in many regions of the world: eculizumab, ravulizumab inebilizumab, and satralizumab. These treatments have significantly improved disease outcomes, offering hope for better management of NMOSD, although challenges remain in early diagnosis, treatment accessibility, and preventing relapses. Further research is essential to refine therapeutic approaches and explore novel targets to enhance patient care.

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KAŁUŻA, Jessica Adriana, BOCHENEK, Oliwia, NOWAK, Aleksandra, KOPER, Mateusz, KONASZCZUK, Adrian, RATYNA, Klaudia, KOZYRA, Oliwia, SZYPUŁA, Zofia, PALUCH, Katarzyna and SKARBEK, Małgorzata. Neuromyelitis Optica Spectrum Disorders – from pathophysiology to treatment. Quality in Sport. Online. 16 September 2024. Vol. 21, p. 54342. [Accessed 1 July 2025]. DOI 10.12775/QS.2024.21.54342.
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Copyright (c) 2024 Jessica Adriana Kałuża, Oliwia Bochenek, Aleksandra Nowak, Mateusz Koper, Adrian Konaszczuk, Klaudia Ratyna, Oliwia Kozyra, Zofia Szypuła, Katarzyna Paluch, Małgorzata Skarbek

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