The annual incidence of Guillain-Barr Syndrome is 1.5 per 100 000. The mortality rate is about 5%, and 10% of patients remain severely disabled one year after neurological onset. Specialist teams, intensive care and rehabilitation are essential for patient management and should be provided in appropriate hospital units. Neither oral nor intravenous corticosteroids are beneficial. Plasma exchange (PE) is the first-line treatment, improving outcome in several randomised, controlled clinical trials. The indications for treatment have been clarified. Two PE sessions are recommended for patients who are able to walk (mild forms), with two additional sessions if they deteriorate. Four sessions are sufficient for patients who are unable to walk unaided (moderate) or who require mechanical ventilation (severe). Further PE sessions are not helpful for patients with more severe disease, or if there is no response. High-dose intravenous immunoglobulins (0.4 g/kg daily for 5 days) and PE are equally effective inpatients with intermediate and severe forms. The choice between the two treatments depends on their respective contraindications and local availability. A trial is underway to determine the indications and optimal dose of IVIg and the optimal number of PE sessions in the different disease severities.
机构:
Hosp Naval Buenos Aires Mayor Pedro Mallo, Div Neurol, RA-1405 Buenos Aires, DF, ArgentinaHosp Naval Buenos Aires Mayor Pedro Mallo, Div Neurol, RA-1405 Buenos Aires, DF, Argentina