ABSTRACT
Background and aims: Myasthenia gravis (MG) is an autoimmune, antibody-mediated disease of the neuromuscular junction causing muscle weakness. Due to immunosuppression and possible respiratory/bulbar muscle weakness, MG patients are at risk for developing severe coronavirus disease 2019 (COVID-19). Methods: Case report. Results: A 62years old patient suffered from bulbar onset generalized MG with acetylcholine receptor antibodies since eight months. He survived two prior MC and was nourished via percutaneous enterogastrostomy (PEG) despite symptomatic treatment and immunosuppression with azathioprine and steroids (severity graded according Myasthenia gravis foundation of America score (MGFA) IVb). He presented with fever, cough and severe generalised muscle weakness. Polymerase chain reaction confirmed COVID-19, ventilatory failure prompted mechanical ventilation (MV). Intravenous immunoglobulins (ivIg) and pyridostigmine i.v. were given. Weaning had to be postponed because of respiratory muscle weakness and septicaemia with Enterobacter cloacae, treated with appriopriate antibiotics. Not until removal of PEG sepsis resolved and plasmapheresis (PLEX) was conducted. The patient reached spontaneous breathing via tracheostomy, but still needed nasogastric tube feeding (MGFA V). Because of missing further progress of bulbar muscle function complement inhibition with eculizumab was administered (900mg 1 x weekly for four weeks, thereafter 1200mg every two weeks). After the second dose tracheostomy and nasogastric tubes could be removed, bulbar and generalized weakness resolved on follow-up (MGFA IIa). Conclusion: Our patient experienced MC triggered by COVID-19 and suffered from prolonged sepsis. After failure of standard treatments (IvIg/PLX) eculizumab was given, followed by marked recovery. The role of complement inhibition for treatment of refractory myasthenic crises should be further studied.