Guillain-Barré syndrome (GBS) was first described in 1916 (Guillain G, 1916) and is approaching its 100th anniversary. Our knowledge of the syndrome has hugely expanded since that time. Once originally considered to be only demyelinating in pathology we now recognise both axonal and demyelinating subtypes. Numerous triggering or antecedent events including infections are recognised and GBS is considered an immunological response to these. GBS is now considered to be a clinical syndrome of an acute inflammatory neuropathy encompassing a number of subtypes with evidence of different immunological mechanisms. Some of these are clearly understood while others remain to be fully elucidated. Complement fixing antibodies against peripheral nerve gangliosides alone and in combination are increasingly recognised as an important mechanism of nerve damage. New antibodies against other nerve antigens such as neurofascin have been recently described. Research databases have been set up to look at factors associated with prognosis and the influence of intravenous immunoglobulin (IvIg) pharmacokinetics in therapy. Exciting new studies are in progress to examine a possible role for complement inhibition in the treatment of the syndrome. 1. Introduction Our understanding of the Guillain-Barré syndrome has improved greatly over the last decade with a much clearer idea of the clinical subtypes of the syndrome and the pathogenesis of some of the rarer variants. 2016 will mark the centenary of the original description by Guillain, Barré and Strohl [1]. They described a rapidly progressive motor disorder associated with absent reflexes and a raised CSF protein in the absence of the expected cerebrospinal fluid (CSF) pleocytosis that characterised poliomyelitis. It became clear, over the ensuing years, that the syndrome varied in severity so that in its severest form it could lead to respiratory paralysis and death [2]. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most frequent subtype in the Western world with a primarily demyelinating pathology and various degrees of secondary axonal damage. Acute motor axonal neuropathy (AMAN) [3] is the next most frequent and appears to be a primary axonal disorder affecting just motor nerves. Axonal variants involving both sensory and motor nerves are much rarer Acute Motor and Sensory Axonal Neuropathy (AMSAN) [3]. Miller Fisher syndrome is generally considered to be allied to GBS although it has a uniquely tight association with anti-GQ1b antibodies. 2. Clinical Features GBS has an incidence of about 1/100,000
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