Clinical Aspects of Guillain-Barré Syndrome: A Review

When, in 1916, Guillain, Barre and Strohl published an account of two soldiers, whose nonfatal ascending paralytic illness was similar in many respects to that described in numerous previous publications (see Haymaker & Kernohan 1949), there was already an extensive literature of cases of ascending flaccid paralysis with sensory symptoms. Landry (1859) reviewed 10 patients with a fatal form of ascending paralysis, some with facial paralysis, but Guillain and his colleagues did not accept these as examples of polyradiculoneuritis because oftheir fatal outcome (Guillain et al. 1916, Guillain 1936a). Guillain (1936a, b) was particularly strict in his definition of the syndrome; for example, he preferred to exclude all cases with a fatal outcome, with a febrile onset, with central manifestations, and with a cerebrospinal fluid (CSF) protein less than 3 g/l. This rigid attitude led to much controversy (see Haymaker & Kernohan 1949) and to attempts to define other similar cases as acute febrile or infective polyneuritis (Osler 1892, Holmes 1917). Although these arguments are principally of historical interest, their legacy can still be discerned in confusion regarding the classification of atypical cases and especially of patients with a subacute onset, but with a relapsing course (Sigwald & Nouailhat 1970, Masucci & Kurtzke 1971). Despite this controversy it must be recognized that the description by Guillain et al. (1916) and Guillain's subsequent work (Guillain et al. 1925, Guillain 1936b, Guillain 1938) clearly delineated the syndrome from other causes of polyneuritis, and from poliomyelitis and syphilitic myelitis. Their description of the raised CSF protein, without a cellular response, was particularly important sincenot only was it useful in diagnosis, but it led to their suggestion that the disorder was a radiculoneuritis, rather than a polyneuritis. Guillain (1936b) listed the main clinical features of the disease, and his account remains an adequate description of the typical subacute disorder. However, several variants of this syndrome are now generally recognized, and Guillain-Barre syndrome may therefore be classified as follows: (1) cases with classical subacute onset; (2) relapsing inflammatory polyradiculoneuropathy; (3) chronic inflammatory polyradiculoneuropathy; (4) cases with central manifestations; (5) cases with other complications; (6) Miller Fisher syndrome.