Myasthenia Gravis and Crisis: Evaluation and Management in the Emergency Department

Jamie Roper, M. Emily Fleming, Brit Long, Alex Koyfman

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background: Myasthenia gravis (MG) is an uncommon autoimmune disorder affecting the neuromuscular junction and manifesting as muscle weakness. A multitude of stressors can exacerbate MG. When symptoms are exacerbated, muscle weakness can be severe enough to result in respiratory failure, a condition known as myasthenic crisis (MC). Objective: This review discusses risk factors, diagnosis, management, and iatrogenic avoidance of MC. Discussion: MC can affect any age, ethnicity, or sex and can be precipitated with any stressor, infection being the most common. MC is a clinical diagnosis defined by respiratory failure caused by exacerbation of MG. Muscle weakness can involve any voluntary muscle. MC can be differentiated from other neuromuscular junction diseases by the presence of normal reflexes, normal sensation, lack of autonomic symptoms, lack of fasciculations, and worsening weakness with repetitive motion. Treatment should target the inciting event and airway support. All acetylcholinesterase inhibitors should be avoided in crisis, including edrophonium testing and corticosteroids initially. Respiratory support can begin with noninvasive positive-pressure ventilation, as this has been successful even in patients with bulbar weakness. If intubation is necessary, consider avoiding paralytics or use a reduced dose of nondepolarizing agents. Conclusions: MC should be in the differential of any patient with muscular weakness and respiratory compromise. Emergency department management of MC should focus on ruling out infection and respiratory support. Strong consideration should be given to beginning with noninvasive positive-pressure ventilation for ventilatory support. Corticosteroids, depolarizing paralytics, and acetylcholinesterase inhibitors should be avoided in patients with MC in the emergency department.

Original languageEnglish (US)
JournalJournal of Emergency Medicine
DOIs
StateAccepted/In press - 2017

Fingerprint

Myasthenia Gravis
Muscle Weakness
Hospital Emergency Service
Neuromuscular Junction Diseases
Positive-Pressure Respiration
Cholinesterase Inhibitors
Respiratory Insufficiency
Adrenal Cortex Hormones
Edrophonium
Fasciculation
Intubation
Respiratory Tract Infections
Reflex
Skeletal Muscle

Keywords

  • Myasthenia gravis
  • Myasthenic crisis
  • Neurology
  • Neuromuscular
  • Respiratory
  • Weakness

ASJC Scopus subject areas

  • Emergency Medicine

Cite this

Myasthenia Gravis and Crisis : Evaluation and Management in the Emergency Department. / Roper, Jamie; Fleming, M. Emily; Long, Brit; Koyfman, Alex.

In: Journal of Emergency Medicine, 2017.

Research output: Contribution to journalArticle

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N2 - Background: Myasthenia gravis (MG) is an uncommon autoimmune disorder affecting the neuromuscular junction and manifesting as muscle weakness. A multitude of stressors can exacerbate MG. When symptoms are exacerbated, muscle weakness can be severe enough to result in respiratory failure, a condition known as myasthenic crisis (MC). Objective: This review discusses risk factors, diagnosis, management, and iatrogenic avoidance of MC. Discussion: MC can affect any age, ethnicity, or sex and can be precipitated with any stressor, infection being the most common. MC is a clinical diagnosis defined by respiratory failure caused by exacerbation of MG. Muscle weakness can involve any voluntary muscle. MC can be differentiated from other neuromuscular junction diseases by the presence of normal reflexes, normal sensation, lack of autonomic symptoms, lack of fasciculations, and worsening weakness with repetitive motion. Treatment should target the inciting event and airway support. All acetylcholinesterase inhibitors should be avoided in crisis, including edrophonium testing and corticosteroids initially. Respiratory support can begin with noninvasive positive-pressure ventilation, as this has been successful even in patients with bulbar weakness. If intubation is necessary, consider avoiding paralytics or use a reduced dose of nondepolarizing agents. Conclusions: MC should be in the differential of any patient with muscular weakness and respiratory compromise. Emergency department management of MC should focus on ruling out infection and respiratory support. Strong consideration should be given to beginning with noninvasive positive-pressure ventilation for ventilatory support. Corticosteroids, depolarizing paralytics, and acetylcholinesterase inhibitors should be avoided in patients with MC in the emergency department.

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