Guillain-Barre syndrome

Background

• Acute polyneuropathy due to immune-mediated peripheral nerve myelin sheath destruction
• Associated with viral or febrile illness, campylobacter infection, or vaccination
• Symptoms at worst 2-4wk after onset, then plateau for 2-4wk, then remit from wks-months

Clinical Features

• Viral illness -> ascending, symmetric weakness or paralysis and loss of DTRs
• May progress to diaphragm resulting in need for mechanical ventilation (33% of pts)
• Autonomic dysfunction occurs in 50% of pts

• Miller-Fisher Syndrome
  • Associated w/ campylobacter infection
  • More likely to be preceded by diarrhea than viral prodrome
  • Consists of ophthalmoplegia and ataxia
  • Weakness is less severe but DESCENDING; disease course milder than classic GBS

Diagnosis

1. Required
   1. Progressive weakness of more than one limb
   2. Areflexia
2. Suggestive
   1. Progression over days to weeks
   2. Recovery beginning 2–4 wk after cessation of progression
   3. Relative symmetry of symptoms
   4. Mild sensory signs and symptoms
   5. CN involvement (Bell's palsy, dysphagia, dysarthria, ophthalmoplegia)
   6. Autonomic dysfunction
      1. Tachycardia, bradycardia, dysrhythmias, wide variations in BP, postural hypotension
      2. Urinary retention
      3. Constipation
      4. Facial flushing
   7. Absence of fever at onset
   8. Cytoalbuminologic dissociation of CSF (high protein (>45) and low WBC count (<10))
   9. Typical findings on electromyogram and nerve conduction studies

DDX

• Weakness

Treatment

1. Intubation indications:
   1. Vital capacity <15mL/kg
   2. PaO2 <70 mm Hg on room air
   3. Bulbar dysfunction (difficulty with breathing, swallowing, or speech)
   4. Aspiration
2. IVIG OR plasmapheresis (provide equivalent but not additive effects)

Disposition

1. Indications for admission to ICU:
   1. Autonomic dysfunction
   2. Bulbar dysfunction
   3. Initial vital capacity <20 mL/kg
   4. Initial negative inspiratory force <–30 cm of water
   5. Decrease of >30% of vital capacity or negative inspiratory force
   6. Inability to ambulate
   7. Treatment with plasmapheresis
   8. Anticipated clinical course requiring mechanical ventilation

Source

Tintinalli