Immunotherapies on Guillain-Barré syndrome and other acute autoimmune neuropathies

Guillain-Barré syndrome (GBS) and other acute autoimmune neuropathies are rare but severe immune-mediated diseases of the peripheral nervous system. Early diagnosis is important for adequate monitoring and treatment, but it can be complicated by the rarity, heterogeneity in clinical presentation, and extensive differential diagnosis. The current proven effective treatments for GBS are intravenous immunoglobulins administered within 2 weeks and plasma exchange within 4 weeks from the onset of weakness. These therapies are immunomodulatory and aim to prevent further damage of peripheral nerves during the acute stage of the disease. Here we provide an overview of the early presentation of GBS and the required diagnostic workup to discriminate GBS from other causes, including other acute forms of autoimmune polyneuropathies. In addition, the current evidence-based immunotherapies for GBS are discussed along with the presumed pathophysiologic mechanisms of action and practical recommendations for the treatment in clinical practice. Despite current treatment, a substantial proportion of patients with GBS have a poor clinical course and outcome. We will present the latest developments regarding potential new treatments that are currently being evaluated and discuss various opportunities to optimize the design of future treatment trials toward a more effective and personalized treatment of GBS.