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What is the pathology of MND

  • In MND there is  loss of large motor neurons in spinal cord & brainstem
  • Gliosis
  • Spheroids are interwoven disorganized neurofilaments in proximal axons
  • Bunina bodies are intracytoplasmic inclusion bodiesi neurons
  • There is  loss of giant Betz cells , other neuronal loss in DRG & Clarkes’ nucleus
  • 1/3 of motoneurons are destroyed before muscle atrophy becomes apparent
  • Peripheral nerve shows secondary degeneration of axons & myelin
  • Surviving motoneurons will develop collaterals branches
  • Atrophy of the degenerated muscles is a prominent finding