Volume 79, Issue 2 pp. 206-216
Research Article

Neuromyelitis optica: Evaluation of 871 attacks and 1,153 treatment courses

Ingo Kleiter MD

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 author
Anna Gahlen BMed

Anna Gahlen BMed

Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Bochum

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Nadja Borisow MD

Nadja Borisow MD

NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Charité University Medicine, and Max Delbrueck Center for Molecular Medicine, Berlin

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Katrin Fischer MD

Katrin Fischer MD

Department of Neurology, Asklepios Clinic Teupitz, Teupitz

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Klaus-Dieter Wernecke PhD

Klaus-Dieter Wernecke PhD

Clinical Research Organisation Sostana and Charité University Medicine, Berlin

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Brigitte Wegner PhD

Brigitte Wegner PhD

Clinical Research Organisation Sostana and Charité University Medicine, Berlin

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Kerstin Hellwig MD

Kerstin Hellwig MD

Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Bochum

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Florence Pache MD

Florence 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

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Klemens Ruprecht MD

Klemens Ruprecht MD

Department of Neurology and Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine, Berlin

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Joachim Havla MD

Joachim Havla MD

Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich

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Markus Krumbholz MD

Markus Krumbholz MD

Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich

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Tania Kümpfel MD

Tania Kümpfel MD

Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich

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Orhan Aktas MD

Orhan Aktas MD

Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf

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Hans-Peter Hartung MD

Hans-Peter Hartung MD

Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf

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Marius Ringelstein MD

Marius Ringelstein MD

Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf

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Christian Geis MD

Christian Geis MD

Department of Neurology, University Hospital Würzburg, Würzburg

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Christoph Kleinschnitz MD

Christoph Kleinschnitz MD

Department of Neurology, University Hospital Würzburg, Würzburg

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Achim Berthele MD

Achim Berthele MD

Department of Neurology, Technical University of Munich, Munich

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Bernhard Hemmer MD

Bernhard Hemmer MD

Department of Neurology, Technical University of Munich and Munich Cluster for Systems Neurology, Munich

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Klemens Angstwurm MD

Klemens Angstwurm MD

Department of Neurology, University Hospital Regensburg, Regensburg

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Jan-Patrick Stellmann MD

Jan-Patrick Stellmann MD

Institute for Neuroimmunology and MS and Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg

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Simon Schuster MD

Simon Schuster MD

Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg

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Martin Stangel MD

Martin Stangel MD

Clinical Neuroimmunology and Neurochemistry, Department of Neurology, Hannover Medical School, Hannover

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Florian Lauda MD

Florian Lauda MD

Department of Neurology, University of Ulm, Ulm

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Hayrettin Tumani MD

Hayrettin Tumani MD

Department of Neurology, University of Ulm, Ulm

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Christoph Mayer MD

Christoph Mayer MD

Department of Neurology, Goethe University Frankfurt, Frankfurt

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Lena Zeltner MD

Lena Zeltner MD

Department of Neurology and Stroke and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen

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Ulf Ziemann MD

Ulf Ziemann MD

Department of Neurology and Stroke and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen

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Ralf Linker MD

Ralf Linker MD

Department of Neurology, Friedrich Alexander University Erlangen-Nuremberg, Erlangen

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Matthias Schwab MD

Matthias Schwab MD

Hans Berger Department of Neurology, Jena University Hospital, Jena

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Martin Marziniak MD

Martin Marziniak MD

Department of Neurology, University of Münster, Münster

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Florian Then Bergh MD

Florian Then Bergh MD

Department of Neurology, University of Leipzig, Leipzig

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Ulrich Hofstadt-van Oy MD

Ulrich Hofstadt-van Oy MD

Department of Neurology, Bayreuth Medical Center, Bayreuth

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Oliver Neuhaus MD

Oliver Neuhaus MD

Department of Neurology, SRH Hospital Sigmaringen, Sigmaringen

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Alexander Winkelmann MD

Alexander Winkelmann MD

Department of Neurology, University of Rostock, Rostock

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Wael Marouf MD

Wael Marouf MD

Department of Neurology, HELIOS Hanse Hospital Stralsund, Stralsund

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Jürgen Faiss MD

Jürgen Faiss MD

Department of Neurology, Asklepios Clinic Teupitz, Teupitz

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Brigitte Wildemann MD

Brigitte Wildemann MD

Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg

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Friedemann Paul MD

Friedemann 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

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Sven Jarius MD

Sven Jarius MD

Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg

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Corinna Trebst MD

Corinna Trebst MD

Department of Neurology, Hannover Medical School, Hannover, Germany

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on behalf of the Neuromyelitis Optica Study Group

on behalf of the Neuromyelitis Optica Study Group

Affiliated members of the Neuromyelitis Optica Study Group are available as an online supplementary file.

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First published: 04 November 2015
Citations: 362

Abstract

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

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