By Damian Baalmann, M.D.
Reviewed by Sara Hocker, M.D.
A 34 year-old male presents to your emergency department in moderate respiratory distress. He is conscious and as the nurses are hooking up monitors, you are able to obtain a history of recent sore throat, followed by increasing weakness in his extremities with progressive dyspnea on exertion. Several years ago, the patient underwent a thymectomy and has since been maintained on prednisone 30-mg/day and pyridostigmine 60-mg Q4H. Vitals: pulse 112, blood pressure 110/80 mmHg and respiratory rate of 26/min. His oxygen saturation is 87% on room air. What are your next steps in management?
Myasthenia Crisis: What is it?
Can differentiate by the presence of cholinergic symptoms including bradycardia, diarrhea, lacrimation, salivation and miosis. Bronchorea may be present in both myasthenic and cholinergic crisis.
With myasthenic crisis, there are several important clinical parameters to assess to help determine whether assisted ventilation is required.
If assisted ventilation is indicated clinically, the patient denies an increase in secretions or difficulty handling their saliva, and the patient is not hypercapnic, a trial of BiPAP may prevent intubation and prolonged ventilation, reduce pulmonary complications and the length of intensive care unit and hospital stay.
If hypercapnia has already developed or there is a pooling of secretions and intubation is necessary, consider the following issues when choosing your paralytic
After airway stabilization, consider some of these interventions (after consulting your friendly neurologist)
Revisiting the Case
Management assessment would include need for emergent intubation, elective intubation, non-invasive ventilation (caution in the setting of excessive secretions) or supplemental oxygen. If intubating, we would be mindful of our paralytic and use more succinylcholine or less rocuronium. We would consider steroids, IVIG or plasmapheresis after consultation with neurology.
Seneviratne J, Mandrekar J, Wijdicks EF, Rabinstein AA. Noninvasive ventilation in myasthenic crisis. Arch Neurol. 2008 Jan;65(1):54-8.
Bird, S. (2012, December 18). Myasthenic crisis. Myasthenic crisis. Retrieved June 18, 2014, from http://www.uptodate.com/contents/myasthenic-crisis?source=machineLearning&search=myasthenic+crisis&selectedTitle=1~33§ionRank=1&anchor=H4#H4
Flower O, Bowles C, Wijdicks E, Weingart SD, Smith WS. Emergency neurological life support: acute non-traumatic weakness. Neurocrit Care. 2012 Sep;17 Suppl 1:S79-95.