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
Authors: Kümpfel Tania , Giglhuber Katrin , Aktas Orhan , Ayzenberg Ilya , Bellmann-Strobl Judith , Häußler Vivien , Havla Joachim , Hellwig Kerstin , Hümmert Martin W. , Jarius Sven , Kleiter Ingo , Klotz Luisa , Krumbholz Markus , Paul Friedemann , Ringelstein Marius , Ruprecht Klemens , Senel Makbule , Stellmann Jan-Patrick , Bergh Florian Then , Trebst Corinna , Tumani Hayrettin , Warnke Clemens , Wildemann Brigitte , Berthele Achim ,   The Neuromyelitis Optica Study Group (NEMOS), Albrecht Philipp , Angstwurm Klemens , Asseyer Susanna , Gomes Ana Beatriz Ayroza Galvao Ribeiro , Bayas Antonios , Behnke Stefanie , Bittner Stefan , Buetow Franziska , Buttmann Mathias , Duchow Ankelien , Engels Daniel , Etgen Thorleif , Fischer Katinka , Frank Benedikt , Gahlen Anna , Gass Achim , Gehring Johannes , Geis Christian , Gold Ralf , Göreci Yasemin , Graf Jonas , Groppa Sergiu , Grothe Matthias , Gutbrod Julia , Guthke Kersten , Haarmann Axel , Hastermann Maria , Hemmer Bernhard , Herfurth Mariella , Herwerth Marina , Hoffmann Frank , Hoffmann Olaf , Hümmert Martin W , Husseini Leila , Junghans Jutta , Kaste Matthias , Kern Peter , Kern Karsten , Kermer Pawel , Kleinschnitz Christoph , Köhler Wolfgang , Körbel Kimberly , Kowarik Markus , Kraemer Markus , Kretschmer Julian , Kurka Natalia , Ladopoulus Theodoros , Lauenstein Ann-Sophie , Laurent Sarah , Lee De-Hyung , Lehrieder Dominik , Leypoldt Frank , Liebetrau Martin , Linker Ralf , Lindenblatt Gero , Lohmann Lisa , Lüssi Felix , Luedemann Peter , Maiworm Michelle , Marziniak Martin , Mayer Christoph , Meister Stefanie , Mering Mathias , Metz Imke , Meuth Sven , Naumann Jasmin , Neuhaus Oliver , Neziraj Tradite , Niederschweiberer Moritz , Niehaus Sabine , Otto Carolin , Pache Florence , Pakeerathan Thivya , Passoke Sarah , Pawlitzki Marc , Pellkofer Hannah , Pompsch Mosche , Pröbstel Anne-Katrin , Pul Refik , Rauer Sebastian , Retzlaff Nele , Riedlinger Arne , Rommer Paulus , Rothhammer Veith , Rostásy Kevin , Rust Rebekka , Ruschil Christoph , Schwab Matthias , Seipelt Maria , Schindler Patrick , Schwake Carolin , Schwarz Patricia , Sommer Claudia , Stefanou Alexander , Sprenger Till , Steinbrecher Andreas , Stephanik Heike , Stoppe Muriel , Stürner Klarissa , Süße Marie , Tarampanis Athanasios , Tauber Simone , Tkachenko Daria , Walter Annette , Wandinger Klaus-Peter , Walz Anna , Weber Martin , Weise Jens , Wickel Jonathan , Wiendl Heinz , Winkelmann Alexander , Yalachkov Yavor , Zettl Uwe , Ziemann Ulf , Zipp Frauke 
Published: September 2023

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.