Programme Hospitalier Recherche Clinique, Institut Pasteur, Inserm, French Public Health Agency.

The posterior reversible encephalopathy syndrome is an increasingly recognised disorder. Most patients have several symptoms; seizures are the most frequent, often multiple or status epilepticus. A combination of seizures, visual disturbance and/or headache, in particular, should lead to an early brain MRI to reveal the typical pattern of bilateral hyperintensities on fluid attenuated inversion recovery imaging, predominantly in the parieto-occipital region. There seem to be many possible triggers, including abrupt arterial hypertension, impaired renal function, pregnancy, immunosuppressive therapies and various inflammatory conditions. The clinical outcome is excellent, with recovery within a few days, while the MRI abnormalities resolve much more slowly. Little is known about the best management. Seizures do not normally progress to chronic epilepsy so antiepileptic drugs should be discontinued after about 3 months.

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.