Neumann, Bernhard, Angstwurm, Klemens, Mergenthaler, Philipp, Kohler, Siegfried, Schoenenberger, Silvia, Boesel, Julian, Neumann, Ursula, Vidal, Amelie, Huttner, Hagen B., Gerner, Stefan T. ORCID: 0000-0001-6020-8290, Thieme, Andrea, Steinbrecher, Andreas, Dunkel, Juliane, Roth, Christian, Schneider, Haucke, Schimmel, Eik, Fuhrer, Hannah, Fahrendorf, Christine, Alberty, Anke, Zinke, Jan, Meisel, Andreas, Dohmen, Christian and Stetefeld, Henning R. (2020). Myasthenic crisis demanding mechanical ventilation: A multicenter analysis of 250 cases. Neurology, 94 (3). S. E299 - 15. PHILADELPHIA: LIPPINCOTT WILLIAMS & WILKINS. ISSN 1526-632X

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Abstract

ObjectiveTo determine demographic characteristics, clinical features, treatment regimens, and outcome of myasthenic crisis (MC) requiring mechanical ventilation (MV).MethodsAnalysis of patients who presented with MC between 2006 and 2015 in a German multicenter retrospective study.ResultsWe 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.ConclusionMortality 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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Neumann, BernhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Angstwurm, KlemensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mergenthaler, PhilippUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kohler, SiegfriedUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schoenenberger, SilviaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Boesel, JulianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neumann, UrsulaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vidal, AmelieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huttner, Hagen B.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Gerner, Stefan T.UNSPECIFIEDorcid.org/0000-0001-6020-8290UNSPECIFIED
Thieme, AndreaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Steinbrecher, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dunkel, JulianeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Roth, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schneider, HauckeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schimmel, EikUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fuhrer, HannahUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fahrendorf, ChristineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Alberty, AnkeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Zinke, JanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Meisel, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dohmen, ChristianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Stetefeld, Henning R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-348463
DOI: 10.1212/WNL.0000000000008688
Journal or Publication Title: Neurology
Volume: 94
Number: 3
Page Range: S. E299 - 15
Date: 2020
Publisher: LIPPINCOTT WILLIAMS & WILKINS
Place of Publication: PHILADELPHIA
ISSN: 1526-632X
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
NONINVASIVE VENTILATION; CLINICAL-FEATURES; RESPIRATORY-FAILURE; PLASMA-EXCHANGE; GRAVIS; MORTALITY; IMMUNOADSORPTION; COMPLICATIONS; PREDICTORS; CAREMultiple languages
Clinical NeurologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/34846

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