Motor Neurone's Disease

Question 1

What are some features that point towards MND?

Answer 1

• Fasciculation
• Absence of sensory signs/symptoms*
• Lower motor neuron signs in arms and upper motor neuron signs in legs
  
  • Lower signs - Decreased muscle tone, weakness and wasting (atrophy) in the muscle(s) supplied by that motor nerve, arreflexia, muscle fasciculations.
  • Upper signs - increased muscle tone, weakness (generally flexors weaker than extensors in the legs and the reverse in the arms - pyramidal pattern of weakness), Increased reflexes, Up-going plantar response and sustained clonus (a few beats is normal).
• Wasting of the small hand muscles/tibialis anterior is common

Question 2

Does MND affect the cerebellar system?

Answer 2

No.

Question 3

Does sphincter dysfunction occur?

Answer 3

Yes but late in the disease.

Question 4

What do nerve conduction studies show in MND?

Answer 4

They are normal.

Question 5

How do you manage MND?

Answer 5

• Riluzole
  
  • Prevents stimulation of glutamate receptors
  • Used mainly in amyotrophic lateral sclerosis
  • Prolongs life by about 3 months
• Respiratory care
  
  • Non-invasive ventilation (usually BIPAP) is used at night
  • Studies have shown a survival benefit of around 7 months
• Prognosis
  
  • poor: 50% of patients die within 3 years

Question 6

MND is any of 5 neurological disorders. What are they?

Answer 6

1. Amyotrophic lateral sclerosis
2. Primary lateral sclerosis
3. Progressive muscular atrophy
4. Progressive bulbar palsy
5. Pseudobulbar palsy.

Question 7

What gender gets MND more men or women?

Answer 7

Men 2:1

Question 8

At what age does MND usually occur?

Answer 8

40-70 years old

50 years is the peak age of onset.