Neuromyelitis optica: evaluation of 871 attacks and 1153 treatment courses

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Item Type:	Article
Title:	Neuromyelitis optica: evaluation of 871 attacks and 1153 treatment courses
Creators Name:	Kleiter, I., Gahlen, A., Borisow, N., Fischer, K., Wernecke, K.D., Wegner, B., Hellwig, K., Pache, F., Ruprecht, K., Havla, J., Krumbholz, M., Kümpfel, T., Aktas, O., Hartung, H.P., Ringelstein, M., Geis, C., Kleinschnitz, C., Berthele, A., Hemmer, B., Angstwurm, K., Stellmann, J.P., Schuster, S., Stangel, M., Lauda, F., Tumani, H., Mayer, C., Zeltner, L., Ziemann, U., Linker, R., Schwab, M., Marziniak, M., Bergh, F.T., Hofstadt-van Oy, U., Neuhaus, O., Winkelmann, A., Marouf, W., Faiss, J., Wildemann, B., Paul, F., 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
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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