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2010-09-21 09:29:56

Motoneuron disease
Show Answers:

  1. what is mixed UMN and LMN motor neurone disease also known as?
    amyotrophic lateral sclerosis
  2. what is the name for pure UMN motoneurone disease?
    primary lateral sclerosis?
  3. what is the name for pure LMN motoneurone disease?
    progressive muscular atrophy
  4. which motoneuron is affected in pseudobulbar palsy?
  5. which motoneuron is affected in bulbar palsy?
  6. where does UMN originate and end?
    • motor region of cerebral cortex ie PMC
    • end: synapses in brainstem (corticobulbar tract) or spinal cord (corticospinal tract)
  7. where is the cell body of LMN? where do LMN axons travel and then synapse?
    • cell body: anterior horn of grey matter or CN nuclei
    • axons: cranial /spinal nerves to the muscle fibre
    • specialised synapse: NMJ
  8. what is the differential diagnosis for any LMN lesion? eg wasting (6 marks)
    • anterior horn cell
    • root
    • brachial plexus
    • nerve
    • NMJ
    • muscle
  9. is anterior horn cell part of LMN or UMN?
  10. what are the 3 main primary diseases of motor neurones (ie anterior horn cell disease)?
    • degeneration: MND, spinal muscular atrophy
    • infection: poliomyelitis
  11. give eg of spinal cord pathology that can affect anterior horn cells?
    • vascular: infarction eg anterior spinal artery
    • mechanical: syringomyelia, tumour
  12. if you think there is a LMN sign eg muscle wasting, what 3 questions need to be asked to get to diagnosis?
    • 1. numbness/tingling/pain? (sensory abnormalities)
    • 2. distribition?
    • 3. UMN signs?
  13. if there IS numbness/tingling or pain, what does that rule out?
    anterior horn/NMJ/myopathy
  14. what is MND?
    relentless degeneration of both UMN and LMNs
  15. what is age of onset in MND?
    late middle age: 50s
  16. what is most common cause of death in MND?
    respiratory failure or pneumonia (aspiration)
  17. what % of MND is familial and which genetic abnormality?
    • 10% familial
    • superoxide dismutase
  18. what 4 muscles/nervous parts does MND spare?
    • extraocular muscles
    • sphincters
    • sensation
    • cognition
  19. which 2 treatable causes have to be excluded before diagnosing MND?
    • cervical spondylosis
    • pure motor neuropathy
  20. if suspect MND, which 3 tests need to be done and why?
    • 1. nerve conduction studies: exclude a neuropathy
    • 2. EMG: confirm DENERVATION and show fasciculations in muscles not thought to be clinically affected
    • 3. MRI/CT: exclude brain/bulbar/spinal cord pathology eg cervical spondylosis or cerebrovascular disease
  21. what is rilozole? benefit?
    • glutamate antagonist
    • only prolongs life by 3-4 months but no proven effect on disability or QOL