The discovery of the Guillain-Barré syndrome and related disorders.

The clinical picture of Guillain–Barré syndrome (GBS) was first clearly described by Landry in 1856 [1]. Although he examined the nerves post-mortem, he did not find any abnormalities. The causative inflammation of the peripheral nerves was not identified until Leyden’s description in 1880 [2]. During the second half of the 19th century, confusion continued whether acute ascending paralysis was ‘‘multiple neuritis’’ or myelitis. In 1916, Guillain, Barré and Strohl reported the characteristic albumino-cytological dissociation in the CSF and undertook graphical recordings of the reduced and delayed tendon reflexes [3,4]. They deduced that the spinal roots were involved and distinguished their syndrome from poliomyelitis. The frequent association of GBS with infections led to the suspicion, never confirmed, that GBS was a viral infection. The occurrence of autoimmune encephalitis following rabies vaccine led Waksman and Adams to experiment with injecting peripheral nerve and adjuvant into animals producing experimental autoimmune neuritis [5]. In 1969 Asbury, Arnason and Adams described severe inflammatory changes in the peripheral nerves of their 19 personal cases of GBS and noted their similarity to those in experimental autoimmune neuritis [6]. This experimental disease is now known to be caused by T helper cells responding to epitopes on myelin proteins. P0, P2 and PMP22 have been the most implicated inducing antigens. The identity of the autoantigen causing the common form of GBS, acute inflammatory demyelinating polyradiculoneuropathy, remains a mystery [7]. The description by Feasby et al. in 1986 of a clinical picture resembling GBS but electrophysiologically and pathologically due to an axonal neuropathy [8] led to the realisation that GBS is a syndrome with more than one pathological substrate. Study of the axonal form of the disease in China by investigators from Johns Hopkins Medical School in the 1990s led to the distinction of a purely motor form of GBS, acute motor axonal neuropathy [9]. Investigation of this homogeneous subtype has enormously enhanced our understanding of autoimmune neuropathy. It is usually preceded by Campylobacter jejuni infection and affected patients have antibodies to gangliosides, especially GM1 and GD1a, whose carbohydrate epitopes resemble those on the axolemma and perisynaptic Schwann cell. Yuki in Japan has produced a rabbit model of the human disease by immunising animals with ganglioside-GM1. Willison in Scotland has produced elegant mouse models and shown that these are caused by complement-fixing antibodies directed against ganglioside-GM1 or GD1a epitopes [10]. Another related neuropathy, Fisher syndrome, consisting of ophthalmoplegia, tendon areflexia and ataxia, turns out to be due to Presse Med. 2013; 42: e177–e179 on line on 2013 Elsevier Masson SAS All rights reserved. www.em-consulte.com/revue/lpm IMMUNE-MEDIATED NEUROPATHIES www.sciencedirect.com