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Ann Neurol. 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.

Author information

1
Department of Neurology, St. Josef-Hospital, Ruhr University Bochum, Bochum.
2
NeuroCure Clinical Research Center and Experimental and Clinical Research Center, Charité University Medicine, and Max Delbrueck Center for Molecular Medicine, Berlin.
3
Department of Neurology, Asklepios Clinic Teupitz, Teupitz.
4
Clinical Research Organisation Sostana and Charité University Medicine, Berlin.
5
Department of Neurology and Clinical and Experimental Multiple Sclerosis Research Center, Charité University Medicine, Berlin.
6
Institute of Clinical Neuroimmunology, Medical Campus Grosshadern, Ludwig Maximilians University, Munich.
7
Department of Neurology, Medical Faculty, Heinrich Heine University Düsseldorf, Düsseldorf.
8
Department of Neurology, University Hospital Würzburg, Würzburg.
9
Department of Neurology, Technical University of Munich, Munich.
10
Department of Neurology, Technical University of Munich and Munich Cluster for Systems Neurology, Munich.
11
Department of Neurology, University Hospital Regensburg, Regensburg.
12
Institute for Neuroimmunology and MS and Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg.
13
Department of Neurology, University Medical Center Hamburg-Eppendorf, Hamburg.
14
Clinical Neuroimmunology and Neurochemistry, Department of Neurology, Hannover Medical School, Hannover.
15
Department of Neurology, University of Ulm, Ulm.
16
Department of Neurology, Goethe University Frankfurt, Frankfurt.
17
Department of Neurology and Stroke and Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen.
18
Department of Neurology, Friedrich Alexander University Erlangen-Nuremberg, Erlangen.
19
Hans Berger Department of Neurology, Jena University Hospital, Jena.
20
Department of Neurology, University of Münster, Münster.
21
Department of Neurology, University of Leipzig, Leipzig.
22
Department of Neurology, Bayreuth Medical Center, Bayreuth.
23
Department of Neurology, SRH Hospital Sigmaringen, Sigmaringen.
24
Department of Neurology, University of Rostock, Rostock.
25
Department of Neurology, HELIOS Hanse Hospital Stralsund, Stralsund.
26
Molecular Neuroimmunology Group, Department of Neurology, University of Heidelberg, Heidelberg.
27
Department of Neurology, Hannover Medical School, Hannover, Germany.

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.

PMID:
26537743
DOI:
10.1002/ana.24554
[Indexed for MEDLINE]

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