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Guillain-Barré syndrome (GBS) has become the major cause of acute neuromuscular paralysis in the Western world since the eradication of polio, with an annual incidence of 1–2 per 100,000. It can affect all ages including children, but becomes increasingly common with age. In the 90 years since the syndrome was first described in World War I soldiers, much progress has been made in understanding its aetiology and treatment. Recently a great deal has changed as we increasingly realise that GBS comprises a group of heterogeneous disorders with likely different immunological pathogeneses.
Clinically, it is useful to divide the syndrome into subtypes rather than lump it all together as one disorder. This leads to appropriate use of investigations, informs evidence based management, and provides a more accurate prognosis for patients.
Despite major advances, GBS, like many other neurological diseases, remains a clinical diagnosis. Laboratory tests help support the diagnosis and exclude other conditions. It is usually fairly easy to recognise the patient who presents with a few days’ history of progressive sensory symptoms, flaccid tetraparesis, and areflexia, perhaps with increasing bulbar weakness. But it can be more difficult to diagnose GBS in the very early stages. By definition the nadir is reached within no more than four weeks of onset, although often this occurs in the first two weeks and in some cases GBS comes on extremely quickly resulting in the need for ventilatory support within 48 hours of symptom onset.
Essential diagnostic criteria include progressive paralysis of more than one limb, and areflexia.1 A diagnosis of GBS is unlikely in the presence of persistent and marked asymmetry of weakness, bladder or bowel dysfunction either at onset or persistently, or with a clear sensory level suggestive of spinal cord pathology.
SUBTYPES OF GBS
It has now become useful to subtype GBS using clinical …