Guillain-Barré Syndrome: Epidemiology, Diagnosis, and Treatment

Abstract

Guillain-Barre syndrome (GBS) is an acute monophasic motor-dominant peripheral neuropathy. A multicenter prospective survey of Guillain-Barre syndrome (GBS) revealed that the axonal types of GBS and Miller Fisher syndrome (MFS), a variant of GBS characterized by ophthalmoplegia and ataxia, are more frequent in Japan than in Western countries. Bickerstaff brainstem encephalitis (BBE) is an acute self-limited disease, characterized by ophthalmoplegia, ataxia, and impaired consciousness and/or pyramidal signs. BBE is considered to form a continuous spectrum with GBS and MFS. A recently performed nationwide survey showed that the annual onset of BBE in Japan was roughly estimated as 100 cases. Diagnosis of GBS can be made by characteristic history and neurological signs, including preceding infection, rapidly progressive symmetric weakness, and decreased tendon reflexes. Nerve conduction studies, cerebrospinal fluid examination, and anti-glycolipid antibody assay are useful for the diagnosis of GBS. Although plasmapheresis and intravenous immunoglobulin (IVIg) are effective therapies for GBS, there are still severe or intractable cases. New therapies such as the second course of IVIg and agents interfering with complement activation are among candidates used for severely affected patients. Prediction of the outcome of each case is important for determining indications of such therapies.