Demyelination- Guillain Barré Syndrome

Question 1

What is it?

Answer 1

• acute paralytic polyneuropathy
• affects peripheral nervous system
• predominantly motor loss

Question 2

features?

Answer 2

• acute, symmetrical, ascending weakness
• but also causes sensory symptoms

Question 3

aetiology?

Answer 3

mainly triggered by an infection. Is particularly associated with:

• campylobacter jejuni
• CMV
• EBV

Question 4

Pathophysiology?

Answer 4

theory of molecular mimicry: the antibodies developed towards antigen of previous infection, also match protein on nerve cells. These can be proteins on the myelin sheath or on the axon itself.

Question 5

Presentation?

Answer 5

• symmetrical ascending weakness, starting at feet and moving up body
• reduced reflexes
• loss of peripheral sensation or neuropathic pain
• involvement of cranial nerves

Question 6

Clinical course?

Answer 6

usually onset is within 4 weeks post infectious trigger

takes 2-4 weeks for symptoms to peak

Question 7

diagnosis?

Answer 7

Diagnosis is clinical- Brighton Criteria can be used. Investigations can aid diagnosis:

• Nerve conduction studies
  
  • reduced signal through nerves
• LP
  
  • raised protein but everything else normal

Question 8

Mx?

Answer 8

• 1st line - IV immunoglobulins / plasma exchange

+supportive care

+ VTE prophylaxis

• as most people die from PE
• if respiratory failure occurs, will need intubation and ventilation and admit to ITU

Question 9

Prognosis?

Answer 9

• 80% recover
• 15% have neurological disability
• 5% die