[Guillain-Barré syndrome]

Abstract

The annual incidence of Guillain-Barré Syndrome is 1.5 per 100000 people. It has an associated mortality of about 5%, with ten percent of patients severely disabled one year after the first onset of neurological signs. To treat this condition, specialist teams, access to intensive care and rehabilitation are essential and should be provided in appropriate hospital units. Neither oral nor intravenous corticosteroids are beneficial. Plasma exchange (PE) is the first line treatment having been found to improve outcomes in several randomised, controlled clinical trials. The indications for treatment have now been clarified. Two PE are recommended in patients who are able to walk (mild) with two additional PE if they deteriorate. In patients unable to walk unaided (moderate), four plasma exchanges are sufficient, likewise in those who require mechanically ventilation (severe form). It is not useful to add further PE in more severe disease or if there is no response. High-dose of intravenous immunoglobulin (0.4 g/kg daily for 5 days) and PE are equally effective in intermediate and severe forms. The choice between the two treatments depends on their respective contra-indications and local availability. A trial is underway to assess the indications and optimal dose of IVIg and number of PE to be used in different severities of disease.