Amyotrophic lateral sclerosis

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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

A man with ALS using a head-mounted laser pointer to comunicate. Note asymetic motor weakness.
  • 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



MRI (axial FLAIR) demonstrates increased T2 signal within the posterior part of the internal capsule, consistent with the diagnosis of ALS.



See Also

External Links


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