Guillain-Barré Syndrome

An 'acute paralytic polyneuropathy' that affects the peripheral nervous system

Aetiology

  • Usually demyelinating or occasionally axonal, and has an immune-mediated, often post-infectious, basis
    • Particularly associated with campylobacter jejuni, cytomegalovirus and Epstein-Barr virus

Pathophysiology

  • Guillain-Barré is thought to occur due to a process called molecular mimicry
  • The B cells of the immune system create antibodies against the antigens on the pathogen that causes the preceding infection
  • These antibodies also match proteins on the nerve cells
  • They may target proteins on the myelin sheath of the motor nerve cell or the nerve axon

Clinical presentation

  • Symptoms usually start within 4 weeks of the preceding infection
  • Symmetrical ascending weakness (starting at the feet and moving up the body)
  • Reduced reflexes
  • There may be peripheral loss of sensation or neuropathic pain
  • It may progress to the cranial nerves and cause facial nerve weakness

Investigations

  • Clinical diagnosis
  • Can be supported by:
    • Nerve conduction studies
    • Lumbar puncture for CSF - will show raised protein with a normal cell count and glucose
  • Bedside spirometry (FVC) monitoring essential due to risk of respiratory failure

Management

  • IV immunoglobulin (first line) or plasma exchange (typically used second line if IV Ig is ineffective)
  • Supportive care
  • VTE prophylaxis (pulmonary embolism is a leading cause of death)
  • In severe cases with respiratory failure patients may need intubation, ventilation and admission to the intensive care unit