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

Ingo Kleiter(St. Josef-Hospital), Anna Gahlen(St. Josef-Hospital), Nadja Borisow(Max Delbrück Center), Katrin Fischer(Klinikum Görlitz), Klaus‐Dieter Wernecke(Charité - Universitätsmedizin Berlin), Brigitte Wegner(Charité - Universitätsmedizin Berlin), Kerstin Hellwig(St. Josef-Hospital), Florence Pache(Max Delbrück Center), Klemens Ruprecht, Joachim Havla(Institute of Neuroimmunology of the Slovak Academy of Sciences), Markus Krumbholz(Institute of Neuroimmunology of the Slovak Academy of Sciences), Tania Kümpfel(Institute of Neuroimmunology of the Slovak Academy of Sciences), Orhan Aktaş(Heinrich Heine University Düsseldorf), Hans‐Peter Hartung(Heinrich Heine University Düsseldorf), Marius Ringelstein(Heinrich Heine University Düsseldorf), Christian Geis, Christoph Kleinschnitz, Achim Berthele(Technical University of Munich), Bernhard Hemmer(Munich Cluster for Systems Neurology), Klemens Angstwurm(University Hospital Regensburg), Jan‐Patrick Stellmann(Universität Hamburg), Simon Schuster(Universität Hamburg), Martin Stangel(Medizinische Hochschule Hannover), Florian Lauda(Universität Ulm), Hayrettin Tumani(Universität Ulm), Christoph Mayer(Goethe University Frankfurt), Lena Zeltner, Ulf Ziemann, Ralf A. Linker(Friedrich-Alexander-Universität Erlangen-Nürnberg), Matthias Schwab(Jena University Hospital), Martin Marziniak, Florian Then Bergh(Leipzig University of Applied Sciences), Ulrich Hofstadt‐van Oy(Bayreuth Medical Center), Oliver Neuhaus(Albstadt-Sigmaringen University), Alexander Winkelmann(University of Rostock), Wael Marouf(University of Applied Sciences Stralsund), Jürgen Faiss(Klinikum Görlitz), Brigitte Wildemann(Heidelberg University), Friedemann Paul(Max Delbrück Center), Sven Jarius(Heidelberg University), Corinna Trebst(Medizinische Hochschule Hannover), on behalf of the Neuromyelitis Optica Study Group(The Stables)
Annals of Neurology
November 4, 2015
10.1002/ana.24554
Cited by 430

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.

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