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Journal Review: Myasthenic crisis
Journal Review: Myasthenic crisis
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Abstract
Myasthenic crisis (MC) is a life-threatening manifestation of myasthenia gravis (MG) defined by respiratory insufficiency that requires the use of invasive or noninvasive ventilation. This is often the result of respiratory muscle weakness but can also be due to bulbar weakness with upper airway collapse. MC occurs in approximately 15%–20% of patients with MG usually within the first 2 to 3 y of the disease course. Many crises have a specific trigger with respiratory infections being most common; however, no specific trigger is found in 30%–40% of patients. MG patients
with a history of MC, severe disease, oropharyngeal weakness, muscle-specific kinase (MuSK) antibodies and thymoma appear to be at higher risk. Most episodes of MC do not occur suddenly, providing a window of opportunity for prevention. Immediate treatment is directed toward airway management and removing any identified triggers. Plasmapheresis is preferred over intravenous immune globulin as the treatments
of choice for MC. The majority of patients are able to be weaned from mechanical ventilation within 1 mo and the outcomes of MC are generally favorable. The mortality rate in United States cohorts is less than 5% and mortality in MC seems to be driven by age and other medical co-morbidities. MC does not appear to affect long-term prognosis as many patients are able to eventually achieve good MG control.

Objectives:
1)Understand the anatomy, physiology, pathogenesis and triggers of myasthenic crisis so as to be able to anticipate and optimally manage them;
2) Recognize possible myasthenic crisis and initiate the appropriate diagnostic measures;
3)Manage myasthenic crisis, including respiratory manifestations.


ACCREDITATION STATEMENT
The AANEM is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.


CREDIT DESIGNATION
The AANEM designates this enduring material for a maximum of 1 AMA PRA Category 1 Credits TM.  Physicians should claim only the credit commensurate with the extent of their participation in the activity. Credit expires 7/1/2026.

DISCLOSURE INFORMATION
Dr. Yuebing Li has consulted for Argenx, Catalyst, Immunovant and UCB Pharma, and has received grant support from Argenx.

FORMAT
PDF
Author
Benjamin Claytor MD; Sung-Min Cho D.O., M.H.; Yuebing Li MD, PhD
Summary
Availability: On-Demand
Expires on Jul 01, 2026
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Non-Member: $25.00
Credit Offered:
1 CME Credit
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