Key Points
- Use the highest‑yield diagnostic test early; do not let testing delay time‑critical therapy.
- Set objective targets and reassess frequently.
- Plan definitive source control or disease‑specific therapy when indicated; document follow‑up and patient education.
Algorithm
- Assess airway/respiratory mechanics; intubate if VC/NIF thresholds crossed or bulbar failure.
- Start IVIG or PLEX; treat triggers (antibiotics for infection, stop offending drugs).
- Restart pyridostigmine when secretions controlled; begin steroid/immunomodulator plan; rehab and swallow evaluation.
Clinical Synopsis & Reasoning
Acute worsening of myasthenia gravis with respiratory or bulbar failure. Monitor VC/NIF, prepare for early intubation, hold cholinesterase inhibitors if excessive secretions, and treat with IVIG or plasma exchange; identify triggers (infection, medications). Corticosteroids and immunomodulators follow stabilization.
Treatment Strategy & Disposition
Stabilize ABCs. Initiate guideline‑concordant first‑line therapy with precise dosing and continuous monitoring. Escalate to advanced/procedural interventions based on explicit failure criteria. Define ICU, step‑down, and ward disposition triggers; involve specialty teams early.
Epidemiology / Risk Factors
- Risk varies by comorbidity and precipitants; see citations for condition‑specific data.
Investigations
| Test | Role / Rationale | Typical Findings | Notes |
|---|
| VC and NIF at bedside | Monitoring | VC <15 mL/kg or NIF >−20 cmH₂O indicates impending failure | Trend frequently |
| ABG/VBG and CXR | Severity/complications | Hypercapnia, pneumonia/atelectasis | Guide support |
| Infection workup and medication review | Etiology | Common triggers include infection, aminoglycosides, Mg | — |
High-Risk & Disposition Triggers
| Trigger | Why it matters | Action |
|---|
| VC <15 mL/kg or NIF worse than −20 cmH2O | Impending respiratory failure | Elective intubation; ICU |
| Bulbar weakness (dysphagia/dysarthria) | Aspiration risk | NPO; airway protection |
| Infection or medication triggers (aminoglycosides, Mg) | Worsening weakness | Stop offending agents; treat infection |
| Cholinergic crisis concern | Mismanagement risk | Hold AChE inhibitor; edrophonium not used; clinical judgment |
| Autonomic dysfunction/arrhythmias | Instability | Telemetry; manage secretions |
Pharmacology
| Medication/Intervention | Mechanism | Onset | Role in Therapy | Limitations |
|---|
| IVIG 2 g/kg over 2–5 days or Plasma exchange (5 exchanges) | Immunotherapy | Days | Rapid improvement | Choose by availability/contraindications |
| Hold or reduce pyridostigmine acutely if bulbar crisis | Anticholinesterase | Hours | Avoid secretions/bradycardia | Restart when stable |
| Corticosteroids and long‑term agents (azathioprine, rituximab) | Immunosuppression | Days‑weeks | Prevent relapse | Neuro follow‑up |
Prognosis / Complications
- Outcome depends on timeliness of diagnosis and definitive therapy; monitor for complications.
Patient Education / Counseling
- Provide red‑flag education, adherence guidance, and explicit return precautions; arrange timely specialty follow‑up.
References
- MGFA/neuromuscular society guidance on myasthenic crisis — Link