Myasthenic crisis demanding mechanical ventilation

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.

Performance of different criteria for refractory myasthenia gravis

“…Although MG can affect bulbar and respiratory muscles requiring intubation and with the risk of death, the outcome of patients with MG has significantly improved over the past decades [1]. Mortality associated with MG was estimated close to 90% in the early 1900s, but currently it is estimated at approximately 10% [2], and 70% to 80% of patients improve with current treatments [1]. Despite the major improvements in MG management, there remains a number of patients who are refractory to treatment, and this proportion varies in the literature from 10% to 20% [3–7].…”

Mortality rates and causes of death in Swedish Myasthenia Gravis patients

“…Despite an increasing number of epidemiological studies on MG [7][8][9][10][11][12] , only a handful of studies have addressed the disease course [13 , 14] , especially regarding overall mortality rates [15][16][17][18] and cause of death [19] in MG patients. Some studies have focused on deaths due to respiratory failure and myasthenic crisis in particular [20][21][22][23][24][25][26][27] . Nevertheless, it remains unknown as to whether there are other frequently occurring causes of death in MG patients.…”

Doctor—Should I get the COVID‐19 vaccine? Infection and immunization in individuals with neuromuscular disorders

“…Exacerbations of myasthenia gravis (MG) are commonly associated with infections 8 . In a recent study of 250 cases of myasthenic crisis requiring mechanical ventilation, the trigger for crisis was an infection in almost half of the patients 9 . In a retrospective cohort of patients under 21 years age and hospitalized with community‐acquired influenza, concomitant NMDs were associated with higher risk of respiratory failure 10 …”