Bernhard Neumann1, Klemens Angstwurm1, Philipp Mergenthaler1, Siegfried Kohler1, Silvia Schönenberger1, Julian Bösel1, Ursula Neumann1, Amelie Vidal1, Hagen B Huttner1, Stefan T Gerner1, Andrea Thieme1, Andreas Steinbrecher1, Juliane Dunkel1, Christian Roth1, Haucke Schneider1, Eik Schimmel1, Hannah Fuhrer1, Christine Fahrendorf1, Anke Alberty1, Jan Zinke1, Andreas Meisel1, Christian Dohmen1, Henning R Stetefeld2. 1. From the Department of Neurology (B.N., K.A., A.V.), University Medical Center Regensburg; NeuroCure Clinical Research Center (P.M., S.K., A.M.) and Departments of Neurology and Experimental Neurology (P.M., A.M.), Charité-Universitätsmedizin Berlin; Berlin Institute of Health (P.M., S.K., A.M.); Department of Neurology (S.S., J.B.), Heidelberg University Hospital; Department of Neurology (J.B.), Klinikum Kassel; Department of Mathematics and Computer Science (U.N.), Philipps-Universität Marburg; Department of Neurology (H.B.H., S.T.G.), University Hospital Erlangen; Department of Neurology (A.T., A.S.), HELIOS Klinikum Erfurt; Department of Neurology (J.D., C.R.), DRK-Kliniken Nordhessen, Kassel; Department of Neurology (H.S., E.S.), University Hospital, Technische Universität Dresden; Department of Neurology (H.S.), Klinikum Augsburg; Department of Neurology (E.S.), Städtisches Klinikum Dresden; Department of Neurology (H.F.), University of Freiburg; Department of Neurology (C.F.), St. Josef-Hospital, Ruhr-University Bochum; Department of Neurology (A.A.), Kliniken Maria Hilf GmbH Mönchengladbach; Hans Berger Department of Neurology (J.Z.), Jena University Hospital; Department of Neurology (C.D., H.R.S.), University of Cologne; and Department of Neurology (C.D.), LVR-Klinik Bonn, Germany. 2. From the Department of Neurology (B.N., K.A., A.V.), University Medical Center Regensburg; NeuroCure Clinical Research Center (P.M., S.K., A.M.) and Departments of Neurology and Experimental Neurology (P.M., A.M.), Charité-Universitätsmedizin Berlin; Berlin Institute of Health (P.M., S.K., A.M.); Department of Neurology (S.S., J.B.), Heidelberg University Hospital; Department of Neurology (J.B.), Klinikum Kassel; Department of Mathematics and Computer Science (U.N.), Philipps-Universität Marburg; Department of Neurology (H.B.H., S.T.G.), University Hospital Erlangen; Department of Neurology (A.T., A.S.), HELIOS Klinikum Erfurt; Department of Neurology (J.D., C.R.), DRK-Kliniken Nordhessen, Kassel; Department of Neurology (H.S., E.S.), University Hospital, Technische Universität Dresden; Department of Neurology (H.S.), Klinikum Augsburg; Department of Neurology (E.S.), Städtisches Klinikum Dresden; Department of Neurology (H.F.), University of Freiburg; Department of Neurology (C.F.), St. Josef-Hospital, Ruhr-University Bochum; Department of Neurology (A.A.), Kliniken Maria Hilf GmbH Mönchengladbach; Hans Berger Department of Neurology (J.Z.), Jena University Hospital; Department of Neurology (C.D., H.R.S.), University of Cologne; and Department of Neurology (C.D.), LVR-Klinik Bonn, Germany. henning.stetefeld@uk-koeln.de.
Abstract
OBJECTIVE: To determine demographic characteristics, clinical features, treatment regimens, and outcome of myasthenic crisis (MC) requiring mechanical ventilation (MV). METHODS: Analysis of patients who presented with MC between 2006 and 2015 in a German multicenter retrospective study. RESULTS: We identified 250 cases in 12 participating centers. Median age at crisis was 72 years. Median duration of MV was 12 days. Prolonged ventilation (>15 days) depended on age (p = 0.0001), late-onset myasthenia gravis (MG), a high Myasthenia Gravis Foundation of America Class before crisis (p = 0.0001 for IVb, odds ratio [OR] = infinite), number of comorbidities (>3 comorbidities: p = 0.002, OR 2.99), pneumonia (p = 0.0001, OR 3.13), and resuscitation (p = 0.0008, OR 9.15). MV at discharge from hospital was necessary in 20.5% of survivors. Patients with early-onset MG (p = 0.0001, OR 0.21), thymus hyperplasia (p = 0.002, OR 0), and successful noninvasive ventilation trial were more likely to be ventilated for less than 15 days. Noninvasive ventilation in 92 cases was sufficient in 38%, which was accompanied by a significantly shorter duration of ventilation (p = 0.001) and intensive care unit (ICU) stay (p = 0.01). IV immunoglobulins, plasma exchange, and immunoadsorption were more likely to be combined sequentially if the duration of MV and the stay in an ICU extended (p = 0.0503, OR 2.05). Patients who received plasma exchange or immunoadsorption as first-line therapy needed invasive ventilation significantly less often (p = 0.003). In-hospital mortality was 12%, which was significantly associated with the number of comorbidities (>3) and complications such as acute respiratory distress syndrome and resuscitation. Main cause of death was multiorgan failure, mostly due to sepsis. CONCLUSION: Mortality and duration of MC remained comparable to previous reports despite higher age and a high disease burden in our study. Prevention and treatment of complications and specialized neurointensive care are the cornerstones in order to improve outcome.
OBJECTIVE: To determine demographic characteristics, clinical features, treatment regimens, and outcome of myasthenic crisis (MC) requiring mechanical ventilation (MV). METHODS: Analysis of patients who presented with MC between 2006 and 2015 in a German multicenter retrospective study. RESULTS: We identified 250 cases in 12 participating centers. Median age at crisis was 72 years. Median duration of MV was 12 days. Prolonged ventilation (>15 days) depended on age (p = 0.0001), late-onset myasthenia gravis (MG), a high Myasthenia Gravis Foundation of America Class before crisis (p = 0.0001 for IVb, odds ratio [OR] = infinite), number of comorbidities (>3 comorbidities: p = 0.002, OR 2.99), pneumonia (p = 0.0001, OR 3.13), and resuscitation (p = 0.0008, OR 9.15). MV at discharge from hospital was necessary in 20.5% of survivors. Patients with early-onset MG (p = 0.0001, OR 0.21), thymus hyperplasia (p = 0.002, OR 0), and successful noninvasive ventilation trial were more likely to be ventilated for less than 15 days. Noninvasive ventilation in 92 cases was sufficient in 38%, which was accompanied by a significantly shorter duration of ventilation (p = 0.001) and intensive care unit (ICU) stay (p = 0.01). IV immunoglobulins, plasma exchange, and immunoadsorption were more likely to be combined sequentially if the duration of MV and the stay in an ICU extended (p = 0.0503, OR 2.05). Patients who received plasma exchange or immunoadsorption as first-line therapy needed invasive ventilation significantly less often (p = 0.003). In-hospital mortality was 12%, which was significantly associated with the number of comorbidities (>3) and complications such as acute respiratory distress syndrome and resuscitation. Main cause of death was multiorgan failure, mostly due to sepsis. CONCLUSION:Mortality and duration of MC remained comparable to previous reports despite higher age and a high disease burden in our study. Prevention and treatment of complications and specialized neurointensive care are the cornerstones in order to improve outcome.