Amyotrophic Lateral Sclerosis (ALS)

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  • 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
  • Involvement of the anterior horn cells

Clinical Features

  • Acute respiratory failure
    • Predicted by forced VC <25 mL/kg or 50% decrease from normal
  • Aspiration pneumonia
  • Trauma related to extremity weakness
  • Asymmetric weakness without sensory findings with mixed upper and lower motor nerve findings

Differential Diagnosis



  1. Nebulized medications
  2. Steroids
  3. Antibiotics for infectious complications
  4. Assisted ventilation / intubation if in respiratory failure and aligned with goals of care

See Also


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