PMID- 37022481 OWN - NLM STAT- MEDLINE DCOM- 20230616 LR - 20230616 IS - 1432-1459 (Electronic) IS - 0340-5354 (Print) IS - 0340-5354 (Linking) VI - 270 IP - 7 DP - 2023 Jul TI - Update on the diagnosis and treatment of neuromyelits optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis. PG - 3341-3368 LID - 10.1007/s00415-023-11634-0 [doi] AB - The term 'neuromyelitis optica spectrum disorders' (NMOSD) is used as an umbrella term that refers to aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica (NMO) and its formes frustes and to a number of closely related clinical syndromes without AQP4-IgG. NMOSD were originally considered subvariants of multiple sclerosis (MS) but are now widely recognized as disorders in their own right that are distinct from MS with regard to immunopathogenesis, clinical presentation, optimum treatment, and prognosis. In part 1 of this two-part article series, which ties in with our 2014 recommendations, the neuromyelitis optica study group (NEMOS) gives updated recommendations on the diagnosis and differential diagnosis of NMOSD. A key focus is on differentiating NMOSD from MS and from myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disease, MOGAD), which shares significant similarity with NMOSD with regard to clinical and, partly, radiological presentation, but is a pathogenetically distinct disease. In part 2, we provide updated recommendations on the treatment of NMOSD, covering all newly approved drugs as well as established treatment options. CI - (c) 2023. The Author(s). FAU - Jarius, Sven AU - Jarius S AD - Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg, Germany. sven.jarius@med.uni-heidelberg.de. FAU - Aktas, Orhan AU - Aktas O AD - Department of Neurology, Medical Faculty, Heinrich Heine University Dusseldorf, Dusseldorf, Germany. FAU - Ayzenberg, Ilya AU - Ayzenberg I AD - Department of Neurology, St. Josef Hospital, Ruhr University Bochum, Bochum, Germany. FAU - Bellmann-Strobl, Judith AU - Bellmann-Strobl J AD - Department of Neurology, Charite-Universitatsmedizin Berlin, corporate member of Freie Universitat Berlin and Humboldt-Universitat zu Berlin, Berlin, Germany. AD - Experimental and Clinical Research Center, a Cooperation between the Max Delbruck Center for Molecular Medicine in the Helmholtz Association and Charite-Universitatsmedizin Berlin, Berlin, Germany. AD - Max Delbruck Center for Molecular Medicine in the Helmholtz Association (MDC), Berlin, Germany. AD - NeuroCure Clinical Research Center, Charite Universitatsmedizin Berlin, corporate member of Freie Universitat Berlin and Humboldt-Universitat zu Berlin, and Berlin Institute of Health, and Max Delbruck Center for Molecular Medicine, Berlin, Germany. FAU - Berthele, Achim AU - Berthele A AD - Department of Neurology, School of Medicine, Technical University Munich, Klinikum rechts der Isar, Munich, Germany. FAU - Giglhuber, Katrin AU - Giglhuber K AD - Department of Neurology, School of Medicine, Technical University Munich, Klinikum rechts der Isar, Munich, Germany. FAU - Haussler, Vivien AU - Haussler V AD - Department of Neurology and Institute of Neuroimmunology and MS (INIMS), University Medical Center Hamburg-Eppendorf, Hamburg, Germany. FAU - Havla, Joachim AU - Havla J AD - Institute of Clinical Neuroimmunology, LMU Hospital, Ludwig-Maximilians-Universitat Munchen, Munich, Germany. AD - Data Integration for Future Medicine (DIFUTURE) Consortium, Ludwig-Maximilians-Universitat Munchen, Munich, Germany. FAU - Hellwig, Kerstin AU - Hellwig K AD - Department of Neurology, St. Josef Hospital, Ruhr University Bochum, Bochum, Germany. FAU - Hummert, Martin W AU - Hummert MW AD - Department of Neurology, Hannover Medical School, Hannover, Germany. FAU - Kleiter, Ingo AU - Kleiter I AD - Department of Neurology, St. Josef Hospital, Ruhr University Bochum, Bochum, Germany. AD - Marianne-Strauss-Klinik, Behandlungszentrum Kempfenhausen fur Multiple Sklerose Kranke, Berg, Germany. FAU - Klotz, Luisa AU - Klotz L AD - Department of Neurology with Institute of Translational Neurology, University of Munster, Munster, Germany. FAU - Krumbholz, Markus AU - Krumbholz M AD - Department of Neurology and Pain Treatment, Immanuel Klinik Rudersdorf, University Hospital of the Brandenburg Medical School Theodor Fontane, Rudersdorf bei Berlin, Germany. AD - Faculty of Health Sciences Brandenburg, Brandenburg Medical School Theodor Fontane, Rudersdorf bei Berlin, Germany. AD - Department of Neurology and Stroke, University Hospital of Tubingen, Tubingen, Germany. FAU - Kumpfel, Tania AU - Kumpfel T AD - Institute of Clinical Neuroimmunology, LMU Hospital, Ludwig-Maximilians-Universitat Munchen, Munich, Germany. FAU - Paul, Friedemann AU - Paul F AD - Department of Neurology, Charite-Universitatsmedizin Berlin, corporate member of Freie Universitat Berlin and Humboldt-Universitat zu Berlin, Berlin, Germany. AD - Experimental and Clinical Research Center, a Cooperation between the Max Delbruck Center for Molecular Medicine in the Helmholtz Association and Charite-Universitatsmedizin Berlin, Berlin, Germany. AD - Max Delbruck Center for Molecular Medicine in the Helmholtz Association (MDC), Berlin, Germany. AD - NeuroCure Clinical Research Center, Charite Universitatsmedizin Berlin, corporate member of Freie Universitat Berlin and Humboldt-Universitat zu Berlin, and Berlin Institute of Health, and Max Delbruck Center for Molecular Medicine, Berlin, Germany. FAU - Ringelstein, Marius AU - Ringelstein M AD - Department of Neurology, Medical Faculty, Heinrich Heine University Dusseldorf, Dusseldorf, Germany. AD - Department of Neurology, Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine University Dusseldorf, Dusseldorf, Germany. FAU - Ruprecht, Klemens AU - Ruprecht K AD - Department of Neurology, Charite-Universitatsmedizin Berlin, corporate member of Freie Universitat Berlin and Humboldt-Universitat zu Berlin, Berlin, Germany. FAU - Senel, Makbule AU - Senel M AD - Department of Neurology, University of Ulm, Ulm, Germany. FAU - Stellmann, Jan-Patrick AU - Stellmann JP AD - Department of Neurology and Institute of Neuroimmunology and MS (INIMS), University Medical Center Hamburg-Eppendorf, Hamburg, Germany. AD - APHM, Hopital de la Timone, CEMEREM, Marseille, France. AD - Aix Marseille Univ, CNRS, CRMBM, Marseille, France. FAU - Bergh, Florian Then AU - Bergh FT AD - Department of Neurology, University of Leipzig, Leipzig, Germany. FAU - Tumani, Hayrettin AU - Tumani H AD - Department of Neurology, University of Ulm, Ulm, Germany. FAU - Wildemann, Brigitte AU - Wildemann B AD - Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg, Germany. FAU - Trebst, Corinna AU - Trebst C AD - Department of Neurology, Hannover Medical School, Hannover, Germany. trebst.corinna@mh-hannover.de. CN - Neuromyelitis Optica Study Group (NEMOS) LA - eng PT - Journal Article PT - Review DEP - 20230406 PL - Germany TA - J Neurol JT - Journal of neurology JID - 0423161 RN - 0 (Myelin-Oligodendrocyte Glycoprotein) RN - 0 (Aquaporin 4) RN - 0 (Immunoglobulin G) RN - 0 (Autoantibodies) SB - IM MH - Humans MH - *Neuromyelitis Optica/diagnosis/therapy MH - Diagnosis, Differential MH - Myelin-Oligodendrocyte Glycoprotein MH - Aquaporin 4 MH - *Multiple Sclerosis/diagnosis/therapy MH - Immunoglobulin G MH - Autoantibodies PMC - PMC10267280 OTO - NOTNLM OT - Aquaporin-4 (AQP4) OT - Autoantibodies OT - Cerebrospinal fluid (CSF) OT - Clinical presentation OT - Diagnosis OT - Diagnostic criteria OT - Differential diagnosis OT - MOG antibody-associated disease (MOGAD) OT - MOG antibody-associated encephalomyelitis (MOG-EM) OT - Magnetic resonance imaging (MRI) OT - Myelin oligodendrocyte glycoprotein (MOG) OT - Myelitis OT - Neuromyelitis optica (NMO) OT - Neuromyelitis optica spectrum disorders (NMOSD) OT - Optic coherence tomography (OCT) OT - Optic neuritis OT - Serology COIS- SJ reports no conflicts of interest. OA has received speaking honoraria and travel grants from Alexion, Almirall, Biogen, Celgene, Merck, Novartis, Roche, and VielaBio. IA has received speaking honoraria and scientific advisory board compensation from Alexion, Roche, Merck Serono, Santhera, and Sanofi Genzyme and research support from Chugai and Diamed. JBS has received speaking honoraria and travel grants from Bayer Healthcare, sanofi-aventis/Genzyme, and Biogen and compensation for serving on a scientific advisory board from Roche. AB has received consulting and/or speaking fees from Alexion, Bayer Healthcare, Biogen, Celgene, and Roche. His institution has received compensations for clinical trials from Alexion, Biogen, Merck, Novartis, Roche, and Sanofi. VH reports no conflicts of interest. JH reports a grant for OCT research from the Friedrich-Baur-Stiftung, personal fees and non-financial support from Merck, Alexion, Novartis, Roche, Santhera, Biogen, Heidelberg Engineering, and Sanofi Genzyme, and non-financial support from the Guthy-Jackson Charitable Foundation; he is partially funded by the German Federal Ministry of Education and Research [grant numbers 01ZZ1603[A-D] and 01ZZ1804[A-H] (DIFUTURE)]. KH reports no conflicts of interest. MH reports no conflicts of interest. IK has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities from Alexion, Almirall, Bayer, Biogen, Hexal, Horizon, Merck, Neuraxpharm, Sanofi, and Roche/Chugai. LK has received compensation for serving on scientific advisory boards from Alexion, Biogen, BMS, Celgene GmbH, Genzyme, Horizon, Janssen, Merck Serono, Novartis, and Roche. She has received speaker honoraria and travel support from Bayer, Biogen, Celgene GmbH, Genzyme, Grifols, Merck Serono, Novartis, Roche, Santhera, and Teva; she receives research support from German Research Foundation, IZKF Munster, IMF Munster, Biogen, Immunic AG, Novartis, and Merck Serono. MK has received travel funding, speaker honoraria, and research support from Bristol Myers Squibb, Merck, Novartis, and Roche. TK has received speaker honoraria and/or personal fees for advisory boards from Novartis Pharma, Roche Pharma, Alexion/Astra Zeneca and Biogen; the Institution she works for has received grant support for her research from Bayer-Schering AG, Novartis and Chugai Pharma. FP has received grants from Guthy Jackson Charitable Foundation, German Research Foundation, German Federal Ministry of Education and Research, Novartis, Bayer, Biogen Idec, Teva, Alexion, Roche, Horizon, Sanofi-Aventis/Genzyme, Merck Serono, Chugai, German Research Council, Werth Stiftung of the City of Cologne, Arthur Arnstein Stiftung Berlin, EU FP7 Framework Program, National Multiple Sclerosis (USA), MedImmune, Shire, and Alexion, and has a patent on Foveal Morphometry pending to Nocturne GmbH; he is Academic Editor at PLoS ONE and Associate Editor of Neurology((R)) Neuroimmunology & Neuroinflammation. MR has received speaker honoraria from Novartis, Bayer Vital GmbH, Roche, Alexion, and Ipsen and travel reimbursement from Bayer Schering, Biogen Idec, Merz, Genzyme, Teva, Roche, and Merck. KR has received research support from Novartis Pharma, Merck Serono, German Ministry of Education and Research, European Union (821283-2), Stiftung Charite (BIH Clinical Fellow Program) and Arthur Arnstein Foundation, speaker honoraria from Bayer, and travel grants from Guthy Jackson Charitable Foundation. MS has received consulting and/or speaker honoraria from Alexion, Bayer, Biogen, Bristol-Myers-Squibb, Merck, Roche, and Sanofi Genzyme. She has received research funding from the Hertha-Nathorff-Program. PS reports no conflict of interest. FTB has received personal compensation for advisory board participation or speaking, and through his institution has received research support for investigator-initiated studies or for attending scientific meetings, from Actelion, Alexion, Bayer-Schering, Biogen, Merck, Novartis, Roche, Sanofi-Genzyme, Teva, UCB, German Research Foundation (DFG), and German Federal Ministry of Education and Research (BMBF). HT has received honoraria for consultation and expert testimony from Alexion Pharma, Bayer, Biogen, Janssen, MERCK, Novartis, Roche, Sanofi Genzyme, and Teva. BW has received grants from the German Ministry of Education and Research, German Research Foundation, Dietmar Hopp Stiftung, Klaus Tschira Stiftung, and Merck, and personal fees from Alexion, Bayer, Biogen, Roche. CT has received honoraria for consultation and expert testimony from Alexion Pharma Germany GmbH, Chugai Pharma Germany GmbH, and Roche Pharma GmbH. EDAT- 2023/04/07 06:00 MHDA- 2023/06/16 06:42 PMCR- 2023/04/06 CRDT- 2023/04/06 11:18 PHST- 2022/10/21 00:00 [received] PHST- 2023/02/18 00:00 [accepted] PHST- 2023/02/17 00:00 [revised] PHST- 2023/06/16 06:42 [medline] PHST- 2023/04/07 06:00 [pubmed] PHST- 2023/04/06 11:18 [entrez] PHST- 2023/04/06 00:00 [pmc-release] AID - 10.1007/s00415-023-11634-0 [pii] AID - 11634 [pii] AID - 10.1007/s00415-023-11634-0 [doi] PST - ppublish SO - J Neurol. 2023 Jul;270(7):3341-3368. doi: 10.1007/s00415-023-11634-0. Epub 2023 Apr 6.