儿童吉兰-巴雷综合征的临床特征及Brighton分层诊断

Clinical features of children with Guillain-Barrésyndrome and the significance of Brighton criteria

摘要:

目的 探讨儿童吉兰-巴雷综合征（GBS）的临床特征及Brighton分层诊断的意义。方法 对72例GBS患儿的临床资料进行回顾性分析，并按Brighton分层诊断进行肯定性分级（1级最高，4级最低）。采用Spearman秩相关评估辅助检查与Brighton分层诊断的相关性。结果 最终共收集GBS患儿72例，平均起病年龄为（98±32）个月。100%（72/72）患儿在4周内肢体无力的严重程度达最高峰，均为腱反射消失或减弱、双侧肢体无力。94%（68/72）患儿神经电生理检查阳性，71%（51/72）患儿脑脊液检查阳性，神经电生理检查阳性率高于脑脊液检查阳性率（P < 0.01）。患儿自发病到进行神经电生理检查的中位间隔时间短于患儿自发病到进行脑脊液检查的中位间隔时间（11 d vs 14 d，P < 0.01）。68%（49/72）患儿达到Brighton分层1级诊断标准，29%（21/72）患儿达到2级诊断标准。神经电生理和脑脊液检查与Brighton分层诊断肯定性分级呈正相关（分别r_s=0.953、0.420，均P < 0.01）。结论 大部分GBS患儿可达到Brighton分层1级诊断，脑脊液和神经电生理检查阳性对提高诊断分级非常重要，疾病早期神经电生理检查比脑脊液检查阳性率更高。

Abstract:

Objective To study the clinical features of children with Guillain-Barrésyndrome(GBS)and the significance of Brighton criteria in childhood GBS.Methods A retrospective analysis was performed on the medical data of 72 children with GBS.Brighton criteria were used for the grading of diagnostic certainty(level 1 as the highest level,and level 4 as the lowest level).A Spearman's rank correlation analysis was used to evaluate the correlation of auxiliary examinations with the level of diagnostic certainty of Brighton criteria.Results A total of 72 children with GBS were enrolled,with a mean age of onset of(98±32)months.All children(100%,72/72)had weakness of bilateral limbs and disappearance or reduction of tendon reflex,and limb weakness reached the highest level of severity within 4 weeks.Of all the 72 children,68(94%)had positive results of neural electrophysiological examination and 51(71%)had positive results of cerebrospinal fluid(CSF)examination,and the positive rate of neural electrophysiological examination was significantly higher than that of CSF examination(P<0.01).The median interval time from disease onset to neural electrophysiological examination was significantly shorter than from disease onset to CSF examination(11 days vs 14 days,P<0.01).Of all the 72 children,49(68%)met Brighton criteria level 1 and 21(29%)met Brighton criteria level 2.Neural electrophysiological examination and CSF examination were positively correlated with the level of diagnostic certainty of Brighton criteria(rs=0.953 and 0.420 respectively,P<0.01).Conclusions Most of the children with GBS meet Brighton criteria level 1,and the positive results of CSF examination and neural electrophysiological examination play an important role in improving the level of diagnostic certainty of Brighton criteria.Neural electrophysiological examination has a higher positive rate than CSF examination in the early stage of the disease.