Determine physician practices regarding management of and recovery from myasthenic crisis.

In discussing the management of myasthenic crisis with neuromuscular experts and clinicians, we noticed large variability in treatment choices. The 2016 consensus guidance for management of myasthenia gravis provides only general recommendations regarding use of acute therapies [intravenous immunoglobulin (IVIg), plasma exchange (PLEX)] and immunosuppressant use in patients with impending/manifest crisis. Literature regarding management of myasthenic crisis and recovery from it is also modest. We therefore wanted to explore clinician’s experience and approach to the in-hospital management of myasthenic crisis and subsequent treatment during the recovery period.

An online questionnaire was created using Google Forms and was piloted at the Annual University of California, Irvine Neuromuscular Colloquium. Subsequent modifications were made to the questionnaire based on the responses and feedback received. The revised questionnaire will be administered through the Rick’s Real Neuromuscular Friends online forum which includes over 2,000 members.

Our preliminary survey revealed interesting results regarding treatment choices for myasthenic crisis. 21/22 respondents were fellowship-trained neurologists with the majority practicing at academic neuromuscular centers. Choice of initial in-hospital treatment for myasthenic crisis varied widely between IVIg or PLEX alone, or in combination with high dose steroids. Similarly, approach to pyridostigmine, prednisone and non-steroidal immunosuppressant dose adjustments was diverse. Majority respondents chose to continue IVIg or PLEX for at least several weeks following myasthenic crisis. Interestingly, nearly half of respondents had considered use of eculizumab for the acute management of crisis or following hospitalization for such.

Our survey indicates that there is considerable variability in the acute treatment of myasthenic crisis, and escalated therapy continues into the post-discharge recovery period. We intend to collate data from a much larger community of clinicians and have this available for presentation at the annual meeting.