Guillain Barre Syndrome

Last edited 02/2023 and last reviewed 03/2023

Guillain-Barre syndrome is an acute, inflammatory, postinfectious polyneuropathy (1,2,3,4,5)

A prodromal malaise with vomiting, headache, fever and limb pains is rapidly surmounted by a progressive and ascending paralysis. This can lead to respiratory dysfunction, and as such, the acute presentation can be a neurological emergency.

GBS has an incidence of about 1/100,000 across several studies in a number of countries

• lifetime risk of GBS is estimated at 1 in 1000 (5)

The incidence of GBS increases in incidence with age and there is a small predominance of males (1)

• 2016 will mark the centenary of the original description by Guillain, Barré and Strohl
  • in their original paper they described a rapidly progressive motor disorder associated with absent reflexes and a raised CSF protein in the absence of the expected cerebrospinal fluid (CSF) pleocytosis that characterised poliomyelitis (2)
    • syndrome varied in severity so that in its severest form it could lead to respiratory paralysis and death
      
      
• GBS can cause severe muscle weakness, and death occurs in about 5% of patients (5)
  • most common subtypes are acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN)
  • approximately 90% of people with GBS in North America and Europe have AIDP
    
    
• subtypes of GBS (3):
• acute inflammatory demyelinating polyradiculoneuropathy (AIDP)
  • the most frequent subtype in the Western world with a primarily demyelinating pathology and various degrees of secondary axonal damage
    • patients with the AIDP subtype of GBS typically have weakness that starts in the legs and spreads to the arms, as well as decreased or absent reflexes (5)
      • in more than 50% of these patients, cranial nerves are affected, which may cause facial weakness, difficulty swallowing, and eye muscle weakness or paralysis
      • 25% to 30% of patients develop severe weakness or paralysis of the respiratory muscles
        
        
• acute motor axonal neuropathy (AMAN)
  • the next most frequent and appears to be a primary axonal disorder affecting just motor nerves
    
    
• Acute Motor and Sensory Axonal Neuropathy (AMSAN)
  • axonal variants involving both sensory and motor nerves are much rarer
    
    
• Miller Fisher syndrome
  • generally considered to be allied to GBS although it has a uniquely tight association with anti-GQ1b antibodies

Management principles:

• treatment options include intravenous immune globulin (IVIG) or plasma exchange
  • 40% to 50% of patients with GBS do not improve within 4 weeks after IVIG or plasma exchange and need prolonged supportive care (5)

Prognosis

• most patients with GBS gradually improve and can have a complete recovery over 6 to 12 months (5)
  • factors associated with a higher risk of death due to GBS include
    • older age,
    • more severe disease, and
    • need for mechanical ventilation

Reference:

• Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology. 2011;36(2):123-133
• Guillain G, Barré J, Strohl A. Sur un syndrome de radiculo-nevrite avec hyperalbuminose du liquide cephalorachidien sans reaction cellulaire. Remarques sur les characteres clinique et graphique des reflexes tendinaux. Bulletins et Memories de la Societe Medicale des Hopitaux de Paris. 1916;40:1462-1470.
• Griffin JW, Li CY, Ho TW, et al. Guillain-Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. Brain. 1995;118(3):577-595.
• Mol Pathol 2001 Dec;54(6):381-5 Guillain Barre syndrome. Winer JB.
• Marcus R. What Is Guillain-Barre Syndrome? JAMA. 2023;329(7):602. doi:10.1001/jama.2022.24232