Guillain-Barre syndrome (GBS)

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Guillain-Barre syndrome is an acute, inflammatory, postinfectious polyneuropathy.

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

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
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
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

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.

Last reviewed 01/2018

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