Clinical features and validation of Brighton criteria in Guillain-Barré syndrome in southern China: retrospective analysis of 1 358 hospitalized patients in four years

Abstract

Objective To analyze the clinical features and validation of Brighton criteria in Guillain-Barre syndrome (GBS) patients from southern China. Methods The clinical data of hospitalized GBS patients from 69 hospitals of 14 provinces/cities in southern China, the area south of the Huaihe River, between 1 January 2013 and 30 September 2016, were collected and analyzed retrospectively, and patients were classified according to the Brighton criteria of case definition, ranging from a highest (defined as level one) to a lowest (level four) level of diagnostic certainty. Results A total of 1 358 GBS patients were collected, including 51 cases with cranial nerve variants, 157 with Miler-Fisher syndrome and 1 150 with classic GBS characterized by flaccid weakness of limbs. Among 1 150 cases of classic GBS, 49.57% (570/1 150) patients had antecedent events, with respiratory infection predominated (71.23%, 406/570); 83.74% (963/1 150) presented limb weakness at onset, 99.21% (1 124/1 133) reached the peak within four weeks, with a score of 3.15±1.16 for Hughes Disability Scale; 99.56%(1 128/1 133) developed bilateral weakness and 95.39% (1 097/1 150) manifested flexia or hyporeflexia; the cerebrospinal fluid showed albuminocytologic dissociation in 80.58% (772/958) patients whose lumbar puncture was performed; demyelinating GBS accounted for 48.14% (401/833) and axonal subtype 18.01% (150/833) respectively in patients with findings of nerve conduction studies available. According to Brighton criteria, the patients were stratified as level one in 44.09% (507/1 150), level two in 45.74% (526/1 150), level three in 7.57% (87/1 150) and level four in 2.61% (30/1 150) of all the patients, and 69.55% (507/729), 28.67% (209/729), 0% (0/729) and 1.78%(13/729), respectively in the patients with complete data (n=729). Conclusions In southern China, demyelinating subtype of GBS is predominant, whereas the proportion of axonal subtype is remarkably lower than that in northern China. The Brighton criteria have a high sensitivity for the diagnosis of GBS in southern China, and examination of cerebrospinal fluid and electrodiagnostic studies are necessary for stratified diagnosis. Key words: Guillain-Barre syndrome; Retrospective studies; Diagnosis; Classification; Brighton criteria