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Guillain-Barré syndrome (GBS) is characterised by a classical triad of progressive motor weakness, areflexia and elevated cerebrospinal fluid (CSF) protein without pleocytosis.1 Landry published the first modern description of an illness likely to be acute inflammatory demyelinating polyneuropathy (AIDP) in 1859. Osler provided a more detailed account of what he called acute febrile polyneuritis in 1892. In 1916, Guillain, Barré and Strohl further enlarged the clinical description and first reported the characteristic CSF finding, albuminocytological dissociation (that is, elevation of CSF protein with normal CSF cell count).1
We consider the pathophysiology, diagnostic features, assessment, differential diagnoses and management of GBS. This article will also outline the spectrum of the disease. Most of the research on GBS has been based on adult or predominantly adult studies, so there has been unavoidable extrapolation with respect to GBS in children and young people.
An annual incidence of 0.5–2 cases per 100 000 has been reported in individuals younger than 18 years of age.2 3 GBS occurs at all ages; the oldest recorded patient was 95 years of age, and the youngest 4 months of age.4 5
Demyelinating and axonal forms of GBS have been described. In the demyelinating form, segmental demyelination of peripheral nerves is found in association with infiltration by inflammatory cells.10 The concept of GBS as a primarily demyelinating neuropathy has been challenged by the discovery of clinically similar cases with severe and probably primary axonal damage,11 12 suggesting the likelihood of two different mechanisms.13 14 Many authors believe that the mechanism of disease involves an abnormal T cell response precipitated by a preceding infection.15 This is thought to give rise to an abnormal immune stimulation. Some of the pathogenic triggers of GBS include viruses such as Epstein-Barr virus,16 cytomegalovirus,16 hepatitis17 …
Competing interests: None.
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