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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.
Glossary
- AChR-Ab=
- acetylcholine receptor antibody;
- CPR=
- cardiopulmonary resuscitation;
- IA=
- immunoadsorption;
- ICD-10=
- International Classification of Diseases–10;
- ICU=
- intensive care unit;
- IVIg=
- IV immunoglobulin;
- LOS-h=
- length of stay in hospital;
- LOS-ICU=
- length of stay in intensive care unit;
- MANOVA=
- multivariate analysis of variance;
- MC=
- myasthenic crisis;
- MG=
- myasthenia gravis;
- MGFA=
- Myasthenia Gravis Foundation of America;
- MLE=
- maximum likelihood estimate;
- MMF=
- mycophenolate mofetil;
- MTX=
- methotrexate;
- MuSK-Ab=
- muscle-specific tyrosine kinase antibody;
- MV=
- mechanical ventilation;
- NICU=
- neurointensive care unit;
- NIV=
- noninvasive ventilation;
- OR=
- odds ratio;
- PE=
- plasma exchange
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* On behalf of the Initiative of German Neurointensive Trial Engagement (IGNITE).
Coinvestigators are listed in appendix 2 at the end of the article.
CME Course: NPub.org/cmelist
- Received February 27, 2019.
- Accepted in final form July 11, 2019.
- © 2019 American Academy of Neurology
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