Motor Neurone Disease

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Motor Neurone Disease

Progressive muscular atrophy, amyotrophic lateral sclerosis

Typical Patient

• 45-65
• M>F
• Mean survival 3 years

History

• NO bladder symptoms

Examination

• Observe
  • wasting
    • small muscles of hand and foot
  • fasciculation
• Neuro
  • tone
    • normal / increased
  • power
    • either segmental (LMN) or pyramidal (UMN)
  • Reflexes
    • exaggerated (usually)
    • Plantar extensor
  • Sensation 
    • NORMAL
  • Coordination normal
  • Also
    • Bulbar Palsy
    • Pseudobulbar Palsy

Cause

• unknown
• 5% familial

Pathology

• degeneration of
  • anterior horn cells
  • corticospinal tracxts
  • motor cortex
  • cranial nerve nuclei
  • corticobulbar tract

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