Anaesthetic implications in patients with disorders of the neuromuscular entity - a problem oriented approach

The diagnosis neuromuscular disorder may apply to numerous rare heterogeneous diseases. While the considerable variation in the clinical picture reflects the wide range of underlying causes, skeletal muscle weakness is usually present. For the anaesthesiologist, management of these patients is always a challenged, since, although infrequent, the disease is associated with a high rate of perioperative complications. Apart from the choice of anaesthetic procedure there are further considerations regarding the overall perioperative management. What pre-operative information needs to be obtained, what intra-operative monitoring is required and how should postoperative monitoring be organized? Additional considerations should include the management of typical complications, the increased risk of bleeding, and anaesthesia during pregnancy or labour Although evidence-based answers are usually not available, an exact diagnosis and careful assessment of the underlying pathophysiological mechanisms may help to optimize anaesthesiological management and reduce the risk for the patient.