Ingo Kleiter1, Anna Gahlen1, Nadja Borisow2, Katrin Fischer3, Klaus-Dieter Wernecke4, Brigitte Wegner4, Kerstin Hellwig1, Florence Pache2,5, Klemens Ruprecht5, Joachim Havla6, Markus Krumbholz6, Tania Kümpfel6, Orhan Aktas7, Hans-Peter Hartung7, Marius Ringelstein7, Christian Geis8, Christoph Kleinschnitz8, Achim Berthele9, Bernhard Hemmer10, Klemens Angstwurm11, Jan-Patrick Stellmann12, Simon Schuster13, Martin Stangel14, Florian Lauda15, Hayrettin Tumani15, Christoph Mayer16, Lena Zeltner17, Ulf Ziemann17, Ralf Linker18, Matthias Schwab19, Martin Marziniak20, Florian Then Bergh21, Ulrich Hofstadt-van Oy22, Oliver Neuhaus23, Alexander Winkelmann24, Wael Marouf25, Jürgen Faiss3, Brigitte Wildemann26, Friedemann Paul2,5, Sven Jarius26, Corinna Trebst27. 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.
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.
Authors: Lidia Stork; David Ellenberger; Tim Beißbarth; Tim Friede; Claudia F Lucchinetti; Wolfgang Brück; Imke Metz Journal: JAMA Neurol Date: 2018-04-01 Impact factor: 18.302
Authors: Sven Jarius; Klemens Ruprecht; Ingo Kleiter; Nadja Borisow; Nasrin Asgari; Kalliopi Pitarokoili; Florence Pache; Oliver Stich; Lena-Alexandra Beume; Martin W Hümmert; Marius Ringelstein; Corinna Trebst; Alexander Winkelmann; Alexander Schwarz; Mathias Buttmann; Hanna Zimmermann; Joseph Kuchling; Diego Franciotta; Marco Capobianco; Eberhard Siebert; Carsten Lukas; Mirjam Korporal-Kuhnke; Jürgen Haas; Kai Fechner; Alexander U Brandt; Kathrin Schanda; Orhan Aktas; Friedemann Paul; Markus Reindl; Brigitte Wildemann Journal: J Neuroinflammation Date: 2016-09-27 Impact factor: 8.322
Authors: Jayne L Chamberlain; Saif Huda; Daniel H Whittam; Marcelo Matiello; B Paul Morgan; Anu Jacob Journal: J Neurol Date: 2019-09-03 Impact factor: 4.849