Amyotrophic Lateral Sclerosis (ALS)

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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