Guillain-Barré Syndrome

Abstract

Guillain-Barre syndrome (GBS) is an acute immune-mediated demyelinating polyneuropathy that often results in severe muscular weakness. Most patients have an antecedent infection within the weeks before the onset of disease. GBS patients generally tend to recover spontaneously, but 20% of patients are still unable to walk independently at 6 months after the onset of disease. General medical care is essential for patients with GBS, but more specific treatment with plasma exchange significantly hastens recovery. High dosage intravenous immunoglobulin (IVIg) is at least as effective as plasma exchange, and may be superior. IVIg has some additional advantages over plasma exchange, because it is widely available, easy to administer and has hardly any contraindications. In clinical trials it was shown that relapse or treatment-related clinical fluctuation occurs in around 10% of GBS patients, irrespective of whether the patients were treated with plasma exchange or IVIg. When a GBS patient deteriorates during or shortly after a course of plasma exchange or IVIg, we recommend not to switch to the other treatment because plasma exchange, and probably IVIg also, is only effective when applied within the first 2 weeks of disease. It is impossible to decide for a single GBS patient whether a particular treatment is effective or not. This is partly due to the large clinical variation in the natural course of disease between individual patients. Subgroups of patients responding particularly favourably to plasma exchange or IVIg have not been identified. Although plasma exchange and IVIg treatment are important advances in the treatment of GBS, a considerable proportion of patients have significant clinical deficit many months or years after the onset of disease. The results of a recently completed nonblinded study suggest that the addition of methylprednisolone to IVIg is even more effective than IVIg alone. Whether this combined treatment is definitely superior to IVIg alone will be investigated in a randomised trial.