Summary
Guillain-Barré syndrome (GBS) is the term used to describe a spectrum of rare post-infectious neuropathies that usually occur in otherwise healthy individuals. GBS is clinically heterogeneous and encompasses acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN), Miller-Fisher syndrome (MFS; see these terms) and some other regional variants. The overall incidence of GBS is between 1.1 and 1.8/100,000/year. In Europe and North America, AIDP is the most frequent form of GBS (accounting for around 90% of cases) and thus the term GBS in general is synonymous with AIDP in Western countries. The axonal forms account for only 3-5% of cases in Western countries but are much more frequent (30%-50% of GBS cases) in Asia and Latin America. In the majority of cases, an infectious disease precedes the onset of limb weakness with Campylobacter jejuni being the most frequently identified initiating event. GBS has also been reported to occur after vaccination or following a surgical intervention. Treatment consists of rapid administration of intravenous immunoglobulin (IVIg) or plasma exchange (PE). Physiotherapy and rehabilitation are also important. The prognosis is variable depending of the form of GBS and ranges from patients with complete recovery, to those who are unable to walk 6 months after the disease onset and to patients in which the disease has a fatal outcome. *Author: Dr P. van Doorn (December 2009)*.
