中国南方吉兰-巴雷综合征的临床特点及Brighton分层诊断：基于四年期间1 358例住院患者的回顾性分析

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

目的 分析中国南方吉兰-巴雷综合征（GBS）患者的临床特点，并探讨其Brighton标准分层诊断的意义。方法 回顾性分析2013年1月1日至2016年9月30日4年期间，淮河以南14个省/市共69家医院GBS住院患者的临床资料，并按Brighton病例定义标准进行诊断的肯定性分级（1级最高、4级最低）。结果 最终共收集GBS患者1 358例，包括脑神经变异型51例、Miler-Fisher综合征157例、肢体无力为主的经典GBS患者1 150例。在经典GBS患者中，49.57%（570/1 150）有前驱事件，其中呼吸道感染占71.23%（406/570）；83.74%（963/1 150）以无力为首发症状；99.21%（1 124/1 133）在4周内无力的严重程度达高峰；无力达高峰时患者的Hughes GBS残障评分为（3.15±1.16）分，99.56%（1 128/1 133）的患者无力呈对称性；95.39%（1 097/1 150）腱反射消失或减弱；在完成腰椎穿刺脑脊液检查的患者中，80.58%（772/958）脑脊液呈蛋白细胞分离；在完成电生理检查的患者中，脱髓鞘性GBS占48.14%（401/833），轴突性GBS占18.01%（150/833）。在所有经典GBS患者中，Brighton分层诊断为1级的患者占44.09%（507/1 150）、2级占45.74%（526/1 150）、3级占7.57%（87/1 150）、4级占2.61%（30/1 150）；其中，在临床资料完善的729例GBS患者中，上述比例分别为69.55%（507/729）、28.67%（209/729）、0%（0/729）、1.78%（13/729）。结论 中国南方GBS患者以脱髓鞘为主要的亚型，而轴突亚型的比例明显低于中国北方；Brighton标准对GBS诊断的敏感度较高，脑脊液和电生理检查对提高诊断分级十分重要。

Objective To analyze the clinical features and validation of Brighton criteria in Guillain-Barré 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.