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Meta-Analysis
. 2016 Feb;79(2):206-16.
doi: 10.1002/ana.24554. Epub 2015 Nov 26.

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

Ingo Kleiter  1 , Anna Gahlen  1 , Nadja Borisow  2 , Katrin Fischer  3 , Klaus-Dieter Wernecke  4 , Brigitte Wegner  4 , Kerstin Hellwig  1 , Florence Pache  2   5 , Klemens Ruprecht  5 , Joachim Havla  6 , Markus Krumbholz  6 , Tania Kümpfel  6 , Orhan Aktas  7 , Hans-Peter Hartung  7 , Marius Ringelstein  7 , Christian Geis  8 , Christoph Kleinschnitz  8 , Achim Berthele  9 , Bernhard Hemmer  10 , Klemens Angstwurm  11 , Jan-Patrick Stellmann  12 , Simon Schuster  13 , Martin Stangel  14 , Florian Lauda  15 , Hayrettin Tumani  15 , Christoph Mayer  16 , Lena Zeltner  17 , Ulf Ziemann  17 , Ralf Linker  18 , Matthias Schwab  19 , Martin Marziniak  20 , Florian Then Bergh  21 , Ulrich Hofstadt-van Oy  22 , Oliver Neuhaus  23 , Alexander Winkelmann  24 , Wael Marouf  25 , Jürgen Faiss  3 , Brigitte Wildemann  26 , Friedemann Paul  2   5 , Sven Jarius  26 , Corinna Trebst  27 ; Neuromyelitis Optica Study Group
Collaborators, Affiliations
Meta-Analysis

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

Ingo Kleiter et al. Ann Neurol. 2016 Feb.

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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