Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part II: Attack therapy and long-term management.

Vivien Häußler, Achim Berthele, Brigitte Wildemann, Clemens Warnke, Hayrettin Tumani, Corinna Trebst, Jan-Patrick Stellmann, Markus Krumbholz, Joachim Havla, Tania Kümpfel, Friedemann Paul, Ingo Kleiter, Sven Jarius, Judith Bellmann-Strobl, Florian Then Bergh, Luisa Klotz, Marius Ringelstein, Orhan Aktas, Katrin Giglhuber, Makbule Senel, Kerstin Hellwig, Ilya Ayzenberg, Martin W Hümmert, Klemens Ruprecht

Journal: Journal of neurology 2024;271(1):141-176

PMID: 37676297

Abstract

This manuscript presents practical recommendations for managing acute attacks and implementing preventive immunotherapies for neuromyelitis optica spectrum disorders (NMOSD), a rare autoimmune disease that causes severe inflammation in the central nervous system (CNS), primarily affecting the optic nerves, spinal cord, and brainstem. The pillars of NMOSD therapy are attack treatment and attack prevention to minimize the accrual of neurological disability. Aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) are a diagnostic marker of the disease and play a significant role in its pathogenicity. Recent advances in understanding NMOSD have led to the development of new therapies and the completion of randomized controlled trials. Four preventive immunotherapies have now been approved for AQP4-IgG-positive NMOSD in many regions of the world: eculizumab, ravulizumab - most recently-, inebilizumab, and satralizumab. These new drugs may potentially substitute rituximab and classical immunosuppressive therapies, which were as yet the mainstay of treatment for both, AQP4-IgG-positive and -negative NMOSD. Here, the Neuromyelitis Optica Study Group (NEMOS) provides an overview of the current state of knowledge on NMOSD treatments and offers statements and practical recommendations on the therapy management and use of all available immunotherapies for this disease. Unmet needs and AQP4-IgG-negative NMOSD are also discussed. The recommendations were developed using a Delphi-based consensus method among the core author group and at expert discussions at NEMOS meetings.

© 2023. The Author(s).

Address: Institute of Clinical Neuroimmunology, LMU Hospital, Ludwig-Maximilians-Universität München, Munich, Germany. [email protected].; Department of Neurology, School of Medicine, Technical University Munich, Klinikum Rechts der Isar, Munich, Germany.; Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf, Germany.; Department of Neurology, St. Josef Hospital, Ruhr University Bochum, Bochum, Germany.; Department of Neurology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; Experimental and Clinical Research Center, a cooperation between the Max Delbrück Center for Molecular Medicine in the Helmholtz Association and Charité-Universitätsmedizin Berlin, Berlin, Germany.; Max Delbrück Center for Molecular Medicine in the Helmholtz Association (MDC), Berlin, Germany.; NeuroCure Clinical Research Center, Charité Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, and Berlin Institute of Health, and Max Delbrück Center for Molecular Medicine, Berlin, Germany.; Department of Neurology and Institute of Neuroimmunology and MS (INIMS), University Medical Center Hamburg-Eppendorf, Hamburg, Germany.; Institute of Clinical Neuroimmunology, LMU Hospital, Ludwig-Maximilians-Universität München, Munich, Germany.; Department of Neurology, Hannover Medical School, Hannover, Germany.; Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg, Germany.; Marianne-Strauß-Klinik, Behandlungszentrum Kempfenhausen für Multiple Sklerose Kranke, Berg, Germany.; Department of Neurology with Institute of Translational Neurology, University of Münster, Münster, Germany.; Department of Neurology and Pain Treatment, Immanuel Klinik Rüdersdorf, University Hospital of the Brandenburg Medical School Theodor Fontane, Rüdersdorf bei Berlin, Germany.; Faculty of Health Sciences Brandenburg, Brandenburg Medical School Theodor Fontane, Rüdersdorf bei Berlin, Germany.; Department of Neurology & Stroke, University Hospital of Tübingen, Tübingen, Germany.; Department of Neurology, Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Düsseldorf, Düsseldorf, Germany.; Department of Neurology, University of Ulm, Ulm, Germany.; APHM, Hopital de la Timone, CEMEREM, Marseille, France.; Aix Marseille University, CNRS, CRMBM, Marseille, France.; Department of Neurology, University of Leipzig, Leipzig, Germany.; Department of Neurology, Faculty of Medicine, University Hospital Cologne, University of Cologne, Cologne, Germany.; Department of Neurology, School of Medicine, Technical University Munich, Klinikum Rechts der Isar, Munich, Germany. [email protected].
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