Zilucoplan in myasthenic crisis refractory to conventional treatment: a report of two cases

Myasthenic crisis (MC) is characterized by severe weakness of respiratory and upper airway muscles. The effectiveness of standard MC treatments may be insufficient, leading to prolonged mechanical ventilation in some patients [1]. Treatment options have recently expanded with the development of novel immunotherapies targeting complement or its subparts. Zilucoplan binds and inhibits the activation of complement component C5. It is administered once daily and has been proven effective in generalized myasthenia gravis (MG) with anti-acetylcholine receptor (AChR) antibodies [2]. However, patients with MC were excluded from these studies. Here we report two cases of MC patients who both showed dramatic improvement with zilucoplan in the intensive care unit (ICU) and abrupt deterioration after discontinuation of treatment. …