Neuromyelitis optica: Evaluation of 871 attacks and 1, 153 treatment courses. Issue 2 (26th November 2015)

• Record Type: Journal Article
• Authors: Kleiter, Ingo; Gahlen, Anna; Borisow, Nadja; Fischer, Katrin; Wernecke, Klaus‐Dieter; Wegner, Brigitte; Hellwig, Kerstin; Pache, Florence; Ruprecht, Klemens; Havla, Joachim; Krumbholz, Markus; Kümpfel, Tania; Aktas, Orhan; Hartung, Hans‐Peter; Ringelstein, Marius; Geis, Christian; Kleinschnitz, Christoph; Berthele, Achim; Hemmer, Bernhard; Angstwurm, Klemens; Stellmann, Jan‐Patrick; Schuster, Simon; Stangel, Martin; Lauda, Florian; Tumani, Hayrettin; Mayer, Christoph; Zeltner, Lena; Ziemann, Ulf; Linker, Ralf; Schwab, Matthias; Marziniak, Martin; Then Bergh, Florian; Hofstadt‐van Oy, Ulrich; Neuhaus, Oliver; Winkelmann, Alexander; Marouf, Wael; Faiss, Jürgen; Wildemann, Brigitte; Paul, Friedemann; Jarius, Sven; Trebst, Corinna;
• Other Names: Albrecht Philipp investigator.; Ayzenberg Ilya investigator.; Bischof Felix investigator.; Böttcher Tobias investigator.; Brettschneider Johannes investigator.; Buttmann Mathias investigator.; Ettrich Barbara investigator.; Gass Achim investigator.; Guthke Kersten investigator.; Hoffmann Frank investigator.; Kaste Matthias investigator.; Kermer Pawel investigator.; Kern Peter investigator.; Köhler Wolfgang investigator.; Kolesilova Ekaterina investigator.; Langel Stefan investigator.; Liebetrau Martin investigator.; Melms Arthur investigator.; Metz Imke investigator.; Münch Christoph investigator.; Niehaus Sabine investigator.; Pellkofer Hannah investigator.; Riedlinger Arne investigator.; Röpke Luise investigator.; Rommer Paulus investigator.; Ruschil Christoph investigator.; Schippling Sven investigator.; Stoppe Muriel investigator.; Tünnerhoff Johannes investigator.; Veauthier Christian investigator.; Walter Annette investigator.; Wandinger Klaus‐Peter investigator.; Weber Martin S. investigator.; Weissert Robert investigator.; Wilke Christian investigator.; Young Kim investigator.; Zentner Christian investigator.; Zettl Uwe investigator.;
• Is Part Of: Annals of neurology. Volume 79:Issue 2(2016:Feb.)
• Page Start: 206
• Page End: 216
• Publication Date: 2015-11-26
• Subjects: Neurology -- Periodicals; Pediatric neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; 616.8
• DOI: 10.1002/ana.24554
• Languages: English
• ISSNs: 0364-5134

Abstract

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. Ann Neurol 2016;79:206–216