Announcements: Neuromuscular section updated with more questions.

  • Admit to NICU q 1 hour neuro checks
  • Place on Telemetry
  • Address Code Status while in ED, may need intubation
  • Check NIF (Negative Inspiratory Force) and FVC q2hrs x 12 hrs then q4hrs. Intubate if NIF < 20 or FVC < 15cc/kg (or 1 liter) or rapid downward trend
  • Do not look at the O2 sat or ABGs as these patients will become hypercarbic before hypoxic. Call RRT at the first sign of respiratory distress
  • Rule out infection with panculture
  • May need Plasmapheresis. Consult IR for placement of the line and consult Lab Medicine for initiation of Plasmapheresis
  • May need IVIg 2gr/kg over 2-5 days. Check IgA level prior to administration of IVIg as it may cause anaphylaxis in patients with IgA deficiency. Give Acetaminophen and Benadryl prior to each IVIg to decrease HA’s
  • Immunomodulatory treatment:
    •  Steroids: may acutely worsen weakness, monitor closely, methylprednisolone 60mg IV daily
    •  If steroid ineffective or contraindicated: azathioprine, mycophenolate mofetil, or cyclosporine
  • Cholinergics: do not give during crisis.
  • FEN: swallow eval, aspiration risk
  • EMG/NCS: decremental response with repetitive stim
  • Avoid: aminoglycosides, erythromycin, azithromycin, tetracyclines, ciprofloxacin, clindamycin, phenytoin, lithium, beta-blockers, procainamide, quinidine and magnesium
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