Rapid response of eculizumab: a rescue therapy for ventilator-dependent refractory myasthenic crisis

Myasthenic crisis (MC) represents the most severe and life-threatening complication of myasthenia gravis (MG). Some patients exhibit refractory responses to conventional immunotherapies, including intravenous immunoglobulin and plasma exchange. This report describes a patient with MC refractory to repetitive high-dose steroids and intravenous immunoglobulin, requiring ventilator support. Within 2 days of eculizumab administration, significant improvement enabled ventilator discontinuation. Subsequent doses further ameliorated limb and pharyngeal weakness, leading to independence. A literature review that identified ten cases reported across five publications highlighted the favorable outcomes achieved with eculizumab in refractory MC, while concomitant respiratory infection was shown to complicate the recovery from MG-related respiratory failure. Although the randomized controlled trials have excluded MC cases, eculizumab has emerged as a promising option for rescue therapy in refractory MC. Larger studies that specifically include MC cases are warranted.