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Neuromyelitis optica: evaluation of 871 attacks and 1153 treatment courses

Item Type:Article
Title:Neuromyelitis optica: evaluation of 871 attacks and 1153 treatment courses
Creators Name:Kleiter, I. and Gahlen, A. and Borisow, N. and Fischer, K. and Wernecke, K.D. and Wegner, B. and Hellwig, K. and Pache, F. and Ruprecht, K. and Havla, J. and Krumbholz, M. and Kümpfel, T. and Aktas, O. and Hartung, H.P. and Ringelstein, M. and Geis, C. and Kleinschnitz, C. and Berthele, A. and Hemmer, B. and Angstwurm, K. and Stellmann, J.P. and Schuster, S. and Stangel, M. and Lauda, F. and Tumani, H. and Mayer, C. and Zeltner, L. and Ziemann, U. and Linker, R. and Schwab, M. and Marziniak, M. and Bergh, F.T. and Hofstadt-van Oy, U. and Neuhaus, O. and Winkelmann, A. and Marouf, W. and Faiss, J. and Wildemann, B. and Paul, F. and Jarius, S. and Trebst, C.
Abstract:Objective: Neuromyelitis optica (NMO) attacks are often severe, difficult to treat, and leave residual deficits. Here, we analyzed the frequency, sequence, and efficacy of therapies used for NMO attacks. Methods: Retrospective review of patient records to assess demographic/diagnostic data, attack characteristics, therapies, and the short-term remission status (complete, CR; partial, 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 (GEE), a patient-based statistical approach. Results: 871 attacks in 185 patients (142 NMO/43 NMOSD, 82% female) were analyzed. The 1153 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's test). Remission rates were higher for isolated optic neuritis vs. isolated myelitis (p<0.001,), and for unilateral vs. 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 (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 vs. HD-S (OR=4.38; p=0.006). Interpretation: Particularly myelitis and bilateral ON have poor remission rates. Escalation of attack therapy improves outcome. PE/IA may increase recovery in isolated myelitis.
Keywords:Germany, Neuromyelitis Optica, Outcome Assessment (Health Care), Registries, Remission Induction, Retrospective Studies
Source:Annals of Neurology
ISSN:0364-5134
Publisher:Wiley (U.S.A.)
Volume:79
Number:2
Page Range:206-216
Date:February 2016
Official Publication:https://doi.org/10.1002/ana.24554
PubMed:View item in PubMed

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