Guillain–Barré syndrome (GBS) is a heterogeneous, potentially life-threatening post infectious disease characterized by rapidly progressive, symmetrical weakness of the extremities. It can affect all ages. Although treatment with intravenous immunoglobulin (IVIg) and plasma exchange (PE) is available, about 25% of patients still develop respiratory insufficiency (requiring ICU admission and artificial ventilation) and many show signs of autonomic dysfunction. After half a year 20% of patients is still unable to walk. Therefore, there is an urgent need for a better treatment. Early 2019 the results of world’s largest randomized placebo-controlled trial on the effect of a second-dose IVIg treatment in GBS patients with a poor prognosis (SID-GBS trial) are expected. Some other, potentially very effective drugs likely will be evaluated in the next coming years. The diagnosis GBS can usually be made on clinical grounds, but it can be difficult especially in young children and in patients presenting with pain. GBS is a heterogeneous disease, in respect of symptoms, severity and prognosis. A lumbar puncture and electrophysiological studies can help to make the diagnosis more likely, and an EMG can differentiate GBS into a demyelinating (AIDP) or axonal (AMAN) subtype. Insight in the immunopathological mechanisms leading to GBS and variants have made an amazing progress over the past 5-10 years. Molecular mimicry of pathogen-borne antigens, leading to generation of cross-reactive antibodies that also target gangliosides, is part of the pathogenesis of GBS; the subtype and severity of the syndrome are partly determined by the nature of the antecedent infection and specificity of such antibodies. These anti-ganglioside antibodies can fix complement and can cause disruption at the node of Ranvier, leading conduction failure and severe weakness. Standard treatment with IVIg or PE is effective, but it is insufficiently effective in many patients. It is to be hoped that the second-dose IVIg trial (SID-GBS), that is currently being conducted in the Netherlands will show that GBS patients with a poor prognosis benefit from a larger increase of serum IgG compared to standard IVIg treatment. Small studies with eculizumab (a complement C5 blocker) indicated that this drug may be effective in GBS. With the use of appropriate prognostic models, like the modified Erasmus GBS outcome scale (mEGOS), it is now possible to predict functional outcome in individual GBS patients, already one week after hospital admission. Identifying patients with a poor prognosis can help to select patients for a more intensified or otherwise more aggressive treatment. It is to be expected that the results of a world-wide prospective study on GBS, IGOS (headed by Prof Bart Jacobs) will additionally gain an enormous amount of relevant data on all different aspects of GBS in the next coming years. These different aspects of GBS, including the presence of GBS with treatment-related fluctuations (GBS-TRF) and the relation with acute-onset CIDP (A-CIDP), that occurs in patients that progress to CIDP will be discussed.
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