Department of Neurology, Hallym University College of Medicine, Seoul, Korea
ABSTRACT
Acquired myasthenia gravis (MG) is an autoimmune disorder of neuromuscular junction transmission clinically manifesting as variable and fluctuating weakness of certain muscles. Myasthenic crisis (MC, defined as respiratory failure with the need for mechanical ventilation) is the most severe complication of MG. Despite the improved treatment of MG, there is a group of patients with MG that suffer from crises. As MC is a rare illness, valid data about the causes, frequency, duration, and course of myasthenic crisis are scarce. Management of MC requires admission of the patient into a neurological ICU and timely institution of an efficient and safe treatment. Despite the growing clinical experience with disease modifying immunotherapy there is no clinical consensus regarding the use of plasma exchange or high dose immunoglobulin treatment in an ICU setting. Every effort should be done to prevent MC and to develop a maintenance management that leads to effective prevention.
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