Neuromyelitis optica: Evaluation of 871 attacks and 1,153 treatment courses
Corresponding Author
Ingo Kleiter MD
Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Bochum
Address correspondence to Dr Kleiter, Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Gudrunstr 56, 44791 Bochum, Germany. E-mail: [email protected]Search for more papers by this authorAnna Gahlen BMed
Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Bochum
Search for more papers by this authorNadja Borisow MD
NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Charité University Medicine, and Max Delbrueck Center for Molecular Medicine, Berlin
Search for more papers by this authorKatrin Fischer MD
Department of Neurology, Asklepios Clinic Teupitz, Teupitz
Search for more papers by this authorKlaus-Dieter Wernecke PhD
Clinical Research Organisation Sostana and Charité University Medicine, Berlin
Search for more papers by this authorBrigitte Wegner PhD
Clinical Research Organisation Sostana and Charité University Medicine, Berlin
Search for more papers by this authorKerstin Hellwig MD
Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Bochum
Search for more papers by this authorFlorence Pache MD
NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Charité University Medicine, and Max Delbrueck Center for Molecular Medicine, Berlin
Department of Neurology and Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine, Berlin
Search for more papers by this authorKlemens Ruprecht MD
Department of Neurology and Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine, Berlin
Search for more papers by this authorJoachim Havla MD
Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich
Search for more papers by this authorMarkus Krumbholz MD
Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich
Search for more papers by this authorTania Kümpfel MD
Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich
Search for more papers by this authorOrhan Aktas MD
Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf
Search for more papers by this authorHans-Peter Hartung MD
Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf
Search for more papers by this authorMarius Ringelstein MD
Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf
Search for more papers by this authorChristian Geis MD
Department of Neurology, University Hospital Würzburg, Würzburg
Search for more papers by this authorChristoph Kleinschnitz MD
Department of Neurology, University Hospital Würzburg, Würzburg
Search for more papers by this authorAchim Berthele MD
Department of Neurology, Technical University of Munich, Munich
Search for more papers by this authorBernhard Hemmer MD
Department of Neurology, Technical University of Munich and Munich Cluster for Systems Neurology, Munich
Search for more papers by this authorKlemens Angstwurm MD
Department of Neurology, University Hospital Regensburg, Regensburg
Search for more papers by this authorJan-Patrick Stellmann MD
Institute for Neuroimmunology and MS and Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg
Search for more papers by this authorSimon Schuster MD
Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg
Search for more papers by this authorMartin Stangel MD
Clinical Neuroimmunology and Neurochemistry, Department of Neurology, Hannover Medical School, Hannover
Search for more papers by this authorFlorian Lauda MD
Department of Neurology, University of Ulm, Ulm
Search for more papers by this authorHayrettin Tumani MD
Department of Neurology, University of Ulm, Ulm
Search for more papers by this authorChristoph Mayer MD
Department of Neurology, Goethe University Frankfurt, Frankfurt
Search for more papers by this authorLena Zeltner MD
Department of Neurology and Stroke and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen
Search for more papers by this authorUlf Ziemann MD
Department of Neurology and Stroke and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen
Search for more papers by this authorRalf Linker MD
Department of Neurology, Friedrich Alexander University Erlangen-Nuremberg, Erlangen
Search for more papers by this authorMatthias Schwab MD
Hans Berger Department of Neurology, Jena University Hospital, Jena
Search for more papers by this authorMartin Marziniak MD
Department of Neurology, University of Münster, Münster
Search for more papers by this authorFlorian Then Bergh MD
Department of Neurology, University of Leipzig, Leipzig
Search for more papers by this authorUlrich Hofstadt-van Oy MD
Department of Neurology, Bayreuth Medical Center, Bayreuth
Search for more papers by this authorOliver Neuhaus MD
Department of Neurology, SRH Hospital Sigmaringen, Sigmaringen
Search for more papers by this authorAlexander Winkelmann MD
Department of Neurology, University of Rostock, Rostock
Search for more papers by this authorWael Marouf MD
Department of Neurology, HELIOS Hanse Hospital Stralsund, Stralsund
Search for more papers by this authorJürgen Faiss MD
Department of Neurology, Asklepios Clinic Teupitz, Teupitz
Search for more papers by this authorBrigitte Wildemann MD
Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg
Search for more papers by this authorFriedemann Paul MD
NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Charité University Medicine, and Max Delbrueck Center for Molecular Medicine, Berlin
Department of Neurology and Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine, Berlin
Search for more papers by this authorSven Jarius MD
Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg
Search for more papers by this authorCorinna Trebst MD
Department of Neurology, Hannover Medical School, Hannover, Germany
Search for more papers by this authoron behalf of the Neuromyelitis Optica Study Group
Affiliated members of the Neuromyelitis Optica Study Group are available as an online supplementary file.
Search for more papers by this authorCorresponding Author
Ingo Kleiter MD
Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Bochum
Address correspondence to Dr Kleiter, Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Gudrunstr 56, 44791 Bochum, Germany. E-mail: [email protected]Search for more papers by this authorAnna Gahlen BMed
Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Bochum
Search for more papers by this authorNadja Borisow MD
NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Charité University Medicine, and Max Delbrueck Center for Molecular Medicine, Berlin
Search for more papers by this authorKatrin Fischer MD
Department of Neurology, Asklepios Clinic Teupitz, Teupitz
Search for more papers by this authorKlaus-Dieter Wernecke PhD
Clinical Research Organisation Sostana and Charité University Medicine, Berlin
Search for more papers by this authorBrigitte Wegner PhD
Clinical Research Organisation Sostana and Charité University Medicine, Berlin
Search for more papers by this authorKerstin Hellwig MD
Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Bochum
Search for more papers by this authorFlorence Pache MD
NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Charité University Medicine, and Max Delbrueck Center for Molecular Medicine, Berlin
Department of Neurology and Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine, Berlin
Search for more papers by this authorKlemens Ruprecht MD
Department of Neurology and Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine, Berlin
Search for more papers by this authorJoachim Havla MD
Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich
Search for more papers by this authorMarkus Krumbholz MD
Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich
Search for more papers by this authorTania Kümpfel MD
Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich
Search for more papers by this authorOrhan Aktas MD
Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf
Search for more papers by this authorHans-Peter Hartung MD
Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf
Search for more papers by this authorMarius Ringelstein MD
Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf
Search for more papers by this authorChristian Geis MD
Department of Neurology, University Hospital Würzburg, Würzburg
Search for more papers by this authorChristoph Kleinschnitz MD
Department of Neurology, University Hospital Würzburg, Würzburg
Search for more papers by this authorAchim Berthele MD
Department of Neurology, Technical University of Munich, Munich
Search for more papers by this authorBernhard Hemmer MD
Department of Neurology, Technical University of Munich and Munich Cluster for Systems Neurology, Munich
Search for more papers by this authorKlemens Angstwurm MD
Department of Neurology, University Hospital Regensburg, Regensburg
Search for more papers by this authorJan-Patrick Stellmann MD
Institute for Neuroimmunology and MS and Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg
Search for more papers by this authorSimon Schuster MD
Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg
Search for more papers by this authorMartin Stangel MD
Clinical Neuroimmunology and Neurochemistry, Department of Neurology, Hannover Medical School, Hannover
Search for more papers by this authorFlorian Lauda MD
Department of Neurology, University of Ulm, Ulm
Search for more papers by this authorHayrettin Tumani MD
Department of Neurology, University of Ulm, Ulm
Search for more papers by this authorChristoph Mayer MD
Department of Neurology, Goethe University Frankfurt, Frankfurt
Search for more papers by this authorLena Zeltner MD
Department of Neurology and Stroke and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen
Search for more papers by this authorUlf Ziemann MD
Department of Neurology and Stroke and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen
Search for more papers by this authorRalf Linker MD
Department of Neurology, Friedrich Alexander University Erlangen-Nuremberg, Erlangen
Search for more papers by this authorMatthias Schwab MD
Hans Berger Department of Neurology, Jena University Hospital, Jena
Search for more papers by this authorMartin Marziniak MD
Department of Neurology, University of Münster, Münster
Search for more papers by this authorFlorian Then Bergh MD
Department of Neurology, University of Leipzig, Leipzig
Search for more papers by this authorUlrich Hofstadt-van Oy MD
Department of Neurology, Bayreuth Medical Center, Bayreuth
Search for more papers by this authorOliver Neuhaus MD
Department of Neurology, SRH Hospital Sigmaringen, Sigmaringen
Search for more papers by this authorAlexander Winkelmann MD
Department of Neurology, University of Rostock, Rostock
Search for more papers by this authorWael Marouf MD
Department of Neurology, HELIOS Hanse Hospital Stralsund, Stralsund
Search for more papers by this authorJürgen Faiss MD
Department of Neurology, Asklepios Clinic Teupitz, Teupitz
Search for more papers by this authorBrigitte Wildemann MD
Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg
Search for more papers by this authorFriedemann Paul MD
NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Charité University Medicine, and Max Delbrueck Center for Molecular Medicine, Berlin
Department of Neurology and Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine, Berlin
Search for more papers by this authorSven Jarius MD
Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg
Search for more papers by this authorCorinna Trebst MD
Department of Neurology, Hannover Medical School, Hannover, Germany
Search for more papers by this authoron behalf of the Neuromyelitis Optica Study Group
Affiliated members of the Neuromyelitis Optica Study Group are available as an online supplementary file.
Search for more papers by this authorAbstract
Objective
Neuromyelitis optica (NMO) attacks often are severe, are difficult to treat, and leave residual deficits. Here, we analyzed the frequency, sequence, and efficacy of therapies used for NMO attacks.
Methods
A retrospective review was made of patient records to assess demographic/diagnostic data, attack characteristics, therapies, and the short-term remission status (complete remission [CR], partial remission [PR], no remission [NR]). Inclusion criteria were NMO according to Wingerchuk's 2006 criteria or aquaporin-4 antibody–positive NMO spectrum disorder (NMOSD). Remission status was analyzed with generalized estimating equations (GEEs), a patient-based statistical approach.
Results
A total of 871 attacks in 185 patients (142 NMO/43 NMOSD, 82% female) were analyzed. The 1,153 treatment courses comprised high-dose intravenous steroids (HD-S; n = 810), plasma exchange (PE; n = 192), immunoadsorption (IA; n = 38), other (n = 80), and unknown (n = 33) therapies. The first treatment course led to CR in 19.1%, PR in 64.5%, and NR in 16.4% of attacks. Second, third, fourth, and fifth treatment courses were given in 28.2%, 7.1%, 1.4%, and 0.5% of attacks, respectively. This escalation of attack therapy significantly improved outcome (p < 0.001, Bowker test). Remission rates were higher for isolated optic neuritis versus isolated myelitis (p < 0.001), and for unilateral versus bilateral optic neuritis (p = 0.020). Isolated myelitis responded better to PE/IA than to HD-S as first treatment course (p = 0.037). Predictors of CR in multivariate GEE analysis were age (odds ratio [OR] = 0.97, p = 0.011), presence of myelitis (OR = 0.38, p = 0.002), CR from previous attack (OR = 6.85, p < 0.001), and first-line PE/IA versus HD-S (OR = 4.38, p = 0.006).
Interpretation
Particularly myelitis and bilateral optic neuritis have poor remission rates. Escalation of attack therapy improves outcome. PE/IA may increase recovery in isolated myelitis. Ann Neurol 2016;79:206–216
Supporting Information
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