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
- Monitor VC/NIF and clinical trajectory; intubate early if declining.
- Start IVIG or plasma exchange; treat triggers; avoid exacerbating drugs.
- Transition to maintenance immunotherapy; plan rehab and follow‑up.
Clinical Synopsis & Reasoning
Crisis is respiratory failure from myasthenia gravis. Monitor VC and NIF closely; intubate early if declining. Treat with IVIG or plasma exchange; manage triggers and adjust anticholinesterase and immunosuppressive therapy. Avoid exacerbating drugs (aminoglycosides, fluoroquinolones, magnesium).
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 serial measurements | Monitoring | Predict impending failure | Guide airway |
| ABG and CXR | Assessment | Hypercapnia, atelectasis or pneumonia | — |
| Infection screen and medication review | Trigger identification | Common precipitants | — |
High-Risk & Disposition Triggers
| Trigger | Why it matters | Action |
|---|
| Impending respiratory failure (VC <15 mL/kg, NIF < -20) | Airway threat | ICU; prepare for intubation |
| Bulbar weakness with aspiration | Pneumonia risk | Airway protection; NPO |
| Sepsis or triggering meds (aminoglycosides, Mg) | Worsening | Stop triggers; treat source |
| Severe autonomic instability | Complications | Close monitoring |
| Poor response to IVIG/plasma exchange | Refractory | Escalate immunosuppression; consult neuroimmunology |
Pharmacology
| Medication/Intervention | Mechanism | Onset | Role in Therapy | Limitations |
|---|
| IVIG 2 g/kg divided over 2–5 days or Plasma Exchange (5 exchanges) | Immunomodulation | Days | Rapid disease control | Choose based on access/contraindications |
| Short‑acting bronchodilators and secretion management | Supportive | Minutes‑hours | Improve ventilation | Avoid magnesium |
| Optimize pyridostigmine dosing and immunosuppression post‑stabilization | Maintenance | Days‑weeks | Prevent recurrence | — |
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
- AAN/neuromuscular crisis management guidance — Link