Severe Guillain-Barré syndrome

Abstract Guillain-Barre syndrome (GBS) is currently the most common cause of acute flaccid paralysis. The disorder is a polyradiculoneuropathy involving mainly motor occasionally sensory and autonomic nerves presenting as acute, usually symmetrical, typically ascending paralysis. Rapidly progressive weakness is the main clinical feature of GBS. It may involve bulbar weakness, respiratory failure, and autonomic nervous system dysfunction. The acute progression of limb weakness, often with sensory and cranial nerve involvement 1–2 weeks after immune stimulation, proceeds to its peak clinical deficit in 2–4 weeks. GBS usually follows a preceding infection or rarely vaccination. Molecular mimicry between microbial and nerve antigens is clearly underlying pathophysiological mechanism behind the development of the disorder. In countries where Zika virus infection is common, an increase in GBS cases has been reported. Treatment of GBS comprises intravenous immunoglobulin (IVIG) or plasma exchange (PE). Approximately one-third of GBS patients are admitted to an intensive care unit, and many require mechanical ventilation. Despite the demonstrated efficacy of PE and IVIG, GBS however remains a disabling disease in a significant proportion of patients, and these treatments have not improved mortality.