Amyotrophic Lateral Sclerosis (ALS)

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Background

  • Progressive muscle atrophy/weakness due to degeneration of upper and lower motor neurons
  • Patients will rarely present to the ED undiagnosed
  • Likely related to mutated superoxide dismutase (SOD1) gene

Clinical Features

  • Acute respiratory failure
    • Predicted by forced VC <25 mL/kg or 50% decrease from normal
  • Aspiration pneumonia
  • Trauma related to extremity weakness

Differential Diagnosis

Weakness

Management

  1. Nebulized medications
  2. Steroids
  3. Antibiotics
  4. Assisted ventilation / intubation

See Also

References

Tintinalli's Emergency Medicine: A Comprehensive Study Guide, 7e (2010), Chapter 167. Chronic Neurologic Disorders