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, Haemophilus influenzae, Mycoplasma pneumoniae, Influenza, HIV
• Also COVID-19, Zika virus, EBV, HSV, VZV, Chikungunya, Escherichia coli, Japanese encephalitis virus
• Non-infectious causes
  • Immunization
  • Surgery, trauma
  • Medications, including TNF-α inhibitors, tacrolimus, suramin, isotretinoin, and immune checkpoint inhibitors
  • 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

Differential Diagnosis

• CIDP
• Thiamine deficiency
• Polyneuropathy, including acute arsenic poisoning and other toxin-induced peripheral polyneuropathy, Lyme disease, tick paralysis, vasculitis, sarcoidosis, porphyria
• Leptomeningeal lymphoma
• Paraneoplastic syndrome
• Spinal cord disorder
• Botulism, myasthenia gravis, Lambert-Eaton syndrome
• Acute polymyositis, dermatomyositis, necrotizing myopathy, critical illness neuropathy

Investigations

• Lumbar puncture, with CSF classically showing albuminocytologic dissociation (elevated protein with normal white count), though neither normal protein levels nor elevated white cells exclude the diagnosis1

Further Reading

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