22 - Inflammatory Mechanisms in Guillain–Barré Syndrome

Abstract

Guillain–Barré syndrome (GBS) is a common cause of acute paralysis that occurs worldwide. In North America and Europe, GBS typically presents as acute inflammatory demyelinating polyradiculoneuropathy (AIDP). AIDP is similar to the syndrome that was originally described. It develops as a relatively acute, progressive paresis of the limbs, occasionally in an ascending pattern, often with sensory symptoms and areflexia. In Northern China, Japan, and Central and South America, the clinical picture is quite different, in that axonal forms occur in 30–47% of patients. Axonal involvement causing pure motor symptoms is known as acute motor axonal neuropathy. When there is both motor and sensory involvement, the condition is known as acute motor and sensory axonal neuropathy (AMSAN). There are similarities clinically between these syndromes and AIDP in that they may all involve the cranial nerves and affect all four limbs and respiratory function. Another variation of GBS was described by Miller Fisher (Miller Fisher syndrome [MFS]) in 1956. This clinical picture consists of a triad of symptoms: ophthalmoplegia, ataxia, and areflexia. The patients described also had elevation of CSF protein levels without cells. They also followed a course of gradual improvement and resolution of clinical symptoms. The similarities between MFS and AIDP clinically strongly suggested that the two conditions were related.