Guillain-Barré Syndrome

Abstract

Guillain-Barré syndrome (GBS) is an acute immune-mediated polyneuropathy. Two thirds of GBS patients are unable to walk at their most severe state of disease. Respiratory insufficiency and autonomic failure are probably the main causes of death. Optimal general care, physiotherapy and the availability of intensive care facilities at short distance are of great importance. Randomized controlled trials show that intravenous immune globulin (IVIg) and plasma exchange (PE) are equally effective in reducing the time to functional recovery. The combination of PE followed by IVIg, is not significantly better than IVIg or PE alone. Corticosteroids alone are not effective in GBS. Approximately 10% of GBS patients deteriorate after initial improvement or stabilization following IVIg or PE treatment, and often require repeated treatment. These "treatment related clinical fluctuations" are more frequently seen in relatively young patients with severe motor and sensory involvement associated with a preceding and possibly ongoing cytomegalovirus infection. Predominantly motor GBS is frequently preceded by a Campylobacter jejeuni infection. Diarrhea prior to GBS is associated with a worse predicted outcome. Further confirmation is needed to determine whether this is especially the case in those patients treated with PE. GBS patients who are unable to walk without assistance, and who are still within their first 2 weeks of the disease, need to be treated. One PE study showed that patients who are only mildly disabled showed positive progress from two plasma exchange sessions. Presently, due to its wide availability, ease of administration, and favorable side-effect profile, IVIg is considered to be the first-line treatment for patients with GBS. Current investigations are trying to determine if IVIg used in combination with methylprednisolone is even more effective in treating GBS.