目的探讨儿童吉兰-巴雷综合征(GBS)电生理变化及临床特征。方法回顾性分析2003年1月至2007年12月首都医科大学附属北京儿童医院住院确诊GBS的77例患儿电生理检测和临床资料。结果 77例GBS患儿中急性炎症性脱髓鞘多发性神经病(AIDP)32例(41.6%),急性运动轴索型GBS(AMAN)34例(44.2%),急性运动感觉轴索型GBS(AMSAN)4例(5.2%),神经失电位型4例(5.2%),难以分类2例和Miller-Fisher综合征1例。77例中男53例,女24例;AIDP型男27例,女5例;AMAN型男18例,女16例。多数病例集中在6-9月份发病,其中AMAN型发病时间有周期性。发病高峰时段为每年7月4日至8月28日。AMAN和AIDP型起病达高峰平均时间分别为(4.94±1.59)d、(7.09±3.17)d;病情高峰时的Hughes功能障碍评分:≤3分者中AIDP型12例,AMAN型9例;≥4分者中AIDP型20例,AMAN型25例。呼吸肌麻痹、颅神经麻痹及植物神经症状方面AMAN型和AIDP型差异无统计学意义(P>0.05);感觉神经受累方面AIDP型明显多于AMAN型,差异有统计学意义(P<0.05)。结论儿童GBS中AMAN和AIDP型发病率相近,AMAN型发病有明显季节性、无性别差异;而AIDP型发病季节性不明显,男性发病率明显高于女性。AMAN型临床进展较AIDP型快,除感觉神经受累方面AIDP型重于AMAN型外,二者在肌无力严重程度、呼吸肌麻痹、颅神经麻痹及植物神经受累方面差异无统计学意义。 Objective To investigate the electrophysiological changes and clinical features of children with Guillain-Barre syndrome (GBS). Methods Retrospective analysis of electrophysiological examination and clinical data of 77 children admitted to Beijing Children’s Hospital of Capital Medical University from January 2003 to December 2007 were retrospectively analyzed. Results There were 32 cases (41.6%) of acute inflammatory demyelinating polyneuropathy (AIDP) in 77 children with GBS, 34 (44.2%) cases of acute motor axonal GBS (AMAN) 4 cases (5.2%) of AMSAN, 4 cases (5.2%) of neural loss potentials, 2 cases difficult to classify and 1 case of Miller-Fisher syndrome. Among 77 cases, 53 were males and 24 were females; 27 were AIDP males and 5 were females; 18 were AMAN males and 16 were females. The majority of cases concentrated in the 6-9 months of onset, of which AMAN type of onset time is periodic. The peak incidence of the annual July 4 to August 28. AMAN and AIDP reached peak mean time (4.94 ± 1.59) d, (7.09 ± 3.17) d respectively; Hughes dysfunction score at peak disease: 12 cases with AIDP ≤ 9 and 9 cases with AMAN ≤ 3, ≥ 4 points AIDP type 20 cases, AMAN type 25 cases. There were no significant differences in AMAN and AIDP between respiratory paralysis, cranial nerve palsy and autonomic symptoms (P> 0.05). There were more AIDP types in sensory nerve involvement than AMAN, with significant difference (P <0.05) . Conclusion The prevalence of AMAN and AIDP in children with GBS is similar. The incidence of AMAN is obviously seasonal and has no gender difference. However, the onset of AIDP is not obvious in season, and the incidence of AMAN in children is obviously higher than that in women. The clinical progress of AMAN was faster than that of AIDP. There was no significant difference in the severity of muscle weakness, respiratory muscle paralysis, cranial nerve palsy and autonomic nerve involvement except AIDP in sensory nerve involvement.