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NeurologyEmergency

Myasthenia Gravis Crisis Management

Myasthenia Gravis Crisis Management: Suspected Myasthenic Crisis → Respiratory Assessment → Elective Intubation → Identify Crisis Trigger → ⚠️ Avoid The...

Pathway Overview

13 steps

Algorithm Steps

13 total

  1. 01Start

    Suspected Myasthenic Crisis

    Patient with MG presenting with worsening weakness or respiratory compromise

  2. 02Decision

    Respiratory Assessment

    Check FVC, NIF, and clinical signs

    • FVC <20 mL/kg = ICU admission
    • FVC <15 mL/kg = consider intubation
    • NIF < -30 cmH2O = respiratory weakness
    • Dyspnea, weak cough, paradoxical breathing
    • Unable to count to 20 in one breath
  3. 03Action

    Elective Intubation

    Early intubation preferred over emergent

    • Avoid succinylcholine (unpredictable response)
    • Reduce non-depolarizing NMB dose by 50%
    • Consider awake fiberoptic if bulbar weakness
    • Plan for difficult weaning
  4. 04Action

    Identify Crisis Trigger

    Search for precipitating factors

    • Infection (most common - 40%)
    • Medication changes/non-compliance
    • Surgery or anesthesia
    • Drugs: aminoglycosides, β-blockers, fluoroquinolones
    • Taper of immunosuppression
  5. 05Warning

    ⚠️ Avoid These Medications

    Can precipitate or worsen crisis

    • Aminoglycosides, fluoroquinolones, macrolides
    • Beta-blockers
    • Magnesium sulfate
    • Neuromuscular blocking agents
    • Statins, IV contrast (iodinated)
  6. 06Decision

    Select Immunomodulatory Treatment

    IVIG vs Plasma Exchange (PLEX)

    • Both equally effective (Class I evidence)
    • PLEX: faster response (2-3 sessions)
    • IVIG: response in 2-3 weeks
    • Choose based on availability and urgency
  7. 07Action

    IVIG

    Intravenous Immunoglobulin

    • 2 g/kg total dose over 2-5 days
    • Pre-treat: acetaminophen, diphenhydramine
    • Monitor for aseptic meningitis, renal failure
    • Contraindicated: IgA deficiency
  8. 08Action

    Manage Cholinesterase Inhibitors

    Consider holding pyridostigmine in crisis

    • May hold during intubation (secretions)
    • Risk of cholinergic crisis if excessive
    • Restart at lower dose when improving
    • 60 mg PO q4-6h typical maintenance
  9. 09Action

    Corticosteroids

    Initiate with caution in crisis

    • Can cause transient worsening first 2 weeks
    • Start low and titrate: prednisone 10-20 mg/day
    • Or high-dose after IVIG/PLEX initiated
    • Target: 1 mg/kg/day (max 100 mg)
  10. 10Outcome

    Clinical Improvement

    Wean vent support, transfer from ICU

    • Continue immunotherapy
    • Optimize long-term immunosuppression
    • Consider thymectomy if indicated
  11. 11Warning

    Refractory Crisis

    Consider additional interventions

    • Repeat IVIG or PLEX
    • Rituximab or eculizumab
    • Prolonged mechanical ventilation
    • Neuromuscular specialist consultation
  12. 12Action

    Plasma Exchange (PLEX)

    Plasmapheresis

    • 5 exchanges over 7-10 days
    • 1.0-1.5 plasma volumes per exchange
    • Requires central venous access
    • Avoid in hemodynamic instability, sepsis
    • Improvement by 2nd-3rd session
  13. Path rejoins step 08Shared downstream outcome
  14. 13Action

    ICU Monitoring

    Serial respiratory assessments

    • Check FVC/NIF every 2-4 hours
    • Continuous pulse oximetry
    • Aspiration precautions
    • HOB elevated 30-45°
  15. Path rejoins step 04Shared downstream outcome

Guideline Source

International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update

Clinical Safety Information

Clinical Decision Support — Not a Substitute for Clinical Judgment

Individual patient factors may require deviation from these recommendations.

Known Limitations

  • Does not address pediatric MG crisis
  • Requires ICU-level monitoring
  • Drug dosing may require adjustment for renal/hepatic function
  • Does not cover all immunosuppressive agents in detail

Contraindicated Populations

pediatric

Applicable Regions

USEUglobal

EU: Similar recommendations; access to PLEX may vary by center

US: IVIG and PLEX equally effective; choice depends on availability

Version 1Next review: 2028-01-01

Frequently Asked Questions

What is the Myasthenia Gravis Crisis Management?

The Myasthenia Gravis Crisis Management is a emergency clinical algorithm for Neurology. It provides a structured decision tree to guide clinical decision-making, based on International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update.

What guideline is the Myasthenia Gravis Crisis Management based on?

This algorithm is based on International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update (DOI: 10.1212/WNL.0000000000011124).

What are the limitations of the Myasthenia Gravis Crisis Management?

Known limitations include: Does not address pediatric MG crisis; Requires ICU-level monitoring; Drug dosing may require adjustment for renal/hepatic function; Does not cover all immunosuppressive agents in detail. Individual patient factors may require deviation from these recommendations.

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