• Slowly progressive, idiopathic neuro-degenerative disorder of upper and lower motor neurons

Risk Factors

  • Age onset 40-70
  • Family hx
  • Head trauma (e.g. military combat related)


  • Dysphagia
  • Laryngospasm
  • SOB
  • Can present w/predominantly bulbar symptoms (25%) including weak, low pitched, monotone, breathy speech, requiring extra effort, speech difficult to understand
  • Others present w/limb dominant symptoms (70%) including weakness, wasting, spasticiity
  • Difficulty swallowing
  • Diminished cough
  • Emotional lability including pathologic laughing
  • No sensory complaints
  • ~1y from symptom onset to diagnosis
  • Avg 3-5y from diagnosis to death (highly variable); shorter for bulbar dz

Physical Exam Findings

  • Spasticity
  • Hyper-reflexia
  • Babinski
  • Bulbar includes tongue fasciculations
  • Tongue muscle wasting
  • Loss of rise in palte w/ahh
  • Weak/breathy voice
  • Limb findings include: weakness, muscle wasting often most apparent in hands
  • Fasciculations of any muscle groups
  • Normal sensation