No abstract

Myasthenia gravis (MG) is the most common disorder of the neuromuscular junction. Myasthenia crisis, defined as respiratory failure requiring mechanical ventilation in MG, is a common life-threatening complication that occurs in approximately 15% to 20% of patients with MG during their life time. The advent of effective mechanical ventilation, specialized neurointensive care units and the widespread use of immunotherapies have substantially altered the prognosis of myasthenic crisis. Early intubation and mechanical ventilation is perhaps the most important step in the management of myasthenic crisis. The authors favor an orotracheal approach for intubation, and placement of small bore duodenal tubes that may help decrease the risk of aspiration and may be more comfortable than regular nasogastric tubes for the patient. Plasma exchange may be more effective than the intravenous immunoglobulin in the treatment of myasthenic crisis involving respiratory failure. A randomized trial is required to confirm the superior efficacy of plasma exchange compared with intravenous immunoglobulin. In the acute setting, the role of immunosuppression and intravenous/intramuscular pyridostigmine and the newer agents such as tacrolimus remains limited and at times controversial. The therapy should be tailored at an individual basis using best clinical judgment.

SUMMARYPrevious studies relating systolic time intervals and measures of cardiac performance have suggested that the time intervals may be useful indices of myocardial contractility. To explore this possibility, systolic times and left ventricular (LV) performance and contractility were measured nearly simultaneously in 14 normal subjects and 56 patients with cardiac disease. Preejection period (PEP) and the ratio of PEP to LV ejection time (LVET) changed significantly with acute inotropic influences (exercise and isoproterenol), were normal in patients with right or left ventricular overloads in whom cardiac index and ejection fraction were depressed but contractile element velocity at peak dP/dt and the Frank-Levinson contractility index were normal, and were significantly abnormal in patients with either clinically evident or occult LV decompensation in whom the measures of contractility were reduced. Correlations of PEP and PEP/LVET with measures of both performance and contractility were insignificant for patients with valvular disease, shunts, or cor pulmonale and significant but weak for the entire series. However, in subjects with either normal left ventricles or cardiac disease confined to the left ventricle, PEP and PEP/LVET exhibited good correlations with measures of pump function and excellent correlations with measures of contractility. These results indicate that the systolic times are a valid measure of contractility which should prove useful in comparing patients with cardiac pathology confined to the LV myocardium and in following patients with extramyocardial hemodynamic lesions of constant severity.

A small proportion of patients who present within 3 hours of symptom onset receive thrombolytic therapy. The observation that patients with more severe neurologic deficits and subsequently worse in-hospital outcomes appear to present early after symptom onset to the hospital may have implications for clinical studies.