Amyotrophic Lateral Sclerosis (ALS)

Summary

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Key Points

• Amyotrophic Lateral Sclerosis (ALS)
  • Also called Lou Gehrig Disease
  • Pathology
    • May be caused by defect in superoxide dismutase 1 (SOD1)
    • Combined UMN (corticobulbar and corticospinal) and LMN (medullary and spinal cord) degeneration
  • Presentation
    • Usually affects adults 50-60 years of age
    • Both UMN and LMN signs
      • LMN deficits
        • Flaccid weakness/paralysis
        • Fasciculations
        • Muscle atrophy
        • Bulbar palsy (dysarthria, dysphagia, tongue atrophy)
      • UMN deficits
        • Spastic (rigid) weakness/paralysis
        • Hyperreflexia
        • Clonus
        • Pseudobulbar palsy (dysarthria, dysphagia, emotional lability)
    • Usually fatal
  • Treatment
    • Riluzole