Guillain-Barré syndrome

Background

Types

• Guillain-Barré syndrome
  • Acute inflammatory demyelinating polyneuropathy (AIDP)
  • Acute motor axonal neuropathy
    • Acute motor-sensory axonal neuropathy
    • Acute motor-conduction-block neuropathy
    • Pharyngeal-cervical-branchial weakness
• Miller Fisher syndrome
  • Incomplete MFS
    • Acute motor-ophthalmoparesis, without ataxia
    • Acute ataxic neyropathy, without ophthalmoparesis
  • Bickerstaff's brainstem encephalitis

Etiology

• Campylobacter jejuni
• Cytomegalovirus
• Epstein-Barr virus
• Zika virus
• HIV
• Non-infectious causes, including immunization, surgery, trauma, and hematopoietic stem cell transplantation

Pathophysiology

• Immune crossreactivity causes autoimmune destruction of either the myelin sheath (AIDP) or the axon itself
• Most common ADAM antibodies are anti-GM1 or anti-GD1a IgG, which recognize gangliosides
• Most common MFS antibody is anti-GQ1b IgG (in 90%)

Epidemiology

• About 1 case per 100,000 people per year in Western countries

Clinical Manifestations

• Ascending paralysis ± sensory involvement
• Usually occurs 1 to 2 weeks after an immune-stimulating even such as illness or vaccination
  • Preceding illness usually a gastrointestinal or respiratory infection
  • Campylobacter jejuni is the most common causative organism identified
  • Rare following influenza vaccination; most notable was the 1976 H1N1 vaccine, with about 1 in 100,000 vaccine recipients developing GBS

Further Reading

• Guillain-Barré syndrome. Lancet. 2016;388:717-727. doi: 10.1016/S0140-6736(16)00339-1