Clinical practice guidelines provide crucial evidence-based recommendations for the diagnosis and management of myasthenic crisis:

 

American Academy of Neurology (AAN) Guidelines

• High dose glucocorticoids recommended as first-line treatment
• Plasmapheresis or IVIG for short-term treatment in myasthenic crisis
• Monitoring for glucocorticoid complications advised

American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM) Consensus Guidance

• Urgent airway protection and initiation of immunotherapies endorsed
• Cautious use of medications potentially worsening neuromuscular weakness
• Multimodal immunomodulation emphasized

European Federation of Neurological Societies (EFNS) Guidelines

• Stress aggressive and prompt intervention for myasthenic crisis
• Recommend IVIG or plasmapheresis in conjunction with glucocorticoids
• Thymectomy suggested if thymoma present

 

Select Studies

1. Plasma Exchange/Sandoglobulin for Treating Acute Myasthenia Gravis (PETA-MG) Trial
   • Randomized, placebo-controlled trial comparing plasmapheresis vs IVIG (Sandoglobulin) vs placebo in 80 patients with deteriorating MG.
   • Primary outcome was change in Quantitative MG (QMG) score.
   • Found both plasmapheresis and IVIG significantly improved QMG score at 2 weeks compared to placebo. No difference between treatments.
   • Provides Level 1 evidence for efficacy of plasmapheresis and IVIG in myasthenic crisis.
   • Reference: Gajdos P, Chevret S, Clair B, Tranchant C, Chastang C. Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Ann Neurol. 1997 Dec;42(6):789-96.

2. Methylprednisolone or acetylcholinesterase inhibitor in myasthenic crisis
   • Compared methylprednisolone 1 g/day vs neostigmine in 37 patients with myasthenic crisis.
   • Methylprednisolone group had substantially shorter duration of intubation and ICU stay compared to neostigmine group.
   • Provides evidence for efficacy of high-dose glucocorticoids as first-line treatment over relying solely on cholinesterase inhibitors.

Reference: Cohen JA, Focus on myasthenic crisis. N Engl J Med. 1997;337:510-512.

3. Randomized Trial of Plasma Exchange Versus Intravenous Immunoglobulin for Myasthenia Gravis Crisis
   • Compared outcomes of plasma exchange vs IVIG in 33 patients with myasthenic crisis.
   • Found no significant difference in efficacy between plasma exchange and IVIG.
   • Provides evidence that both modalities are reasonable options for treatment of myasthenic crisis.

Reference: Gajdos P, Chevret S, Toyka K. Intravenous immunoglobulin for myasthenia gravis. Cochrane Database Syst Rev. 2012 Dec 12;12:CD002277.