儿童吉兰-巴雷综合征电生理检测及临床特征分析

摘要：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。

The electrophysiology and clinical characteristics of the children with Guillain-Barré syndrome.

Abstract：Objective　To study the epidemiology，clinical and electrophysiological characteristics of the children with Guillain-Barré syndrome. Methods　Retrospectively analyze the electrophysiological and clinical data of 77 children wtih GBS in our hospital. Results　According to clinical and electrophysiologic findings，32 patients manifested acute inflammatory demyelinating polyradiculoneuropathy（AIDP）， 34 had acute motor axonal neuropathy（AMAN），4 had acute motor and sensory axonal neuropathy（ASMAN）， 4 were inexcitable， 2 were unclassified and 1 had Miller-Fisher syndrome. The 77 patients included 53 boys and 25 girls.There were 27 boys and 5 girls in AIDP group and 18 boys and 16 girls in AMAN group. The AMAN group wer clearly seasonal （June 4 to August 28） .The mean time from the onset of illness to nadir was 7.09±3.17 days in the AIDP group and 4.94±1.59 days in the AMAN group. The mean disability score at nadir by the Hughes scale of 12 cases in AIDP group and 9 cases in AMAN group was ≤3，and 20 cases in AIDP group and 25 cases in AMAN group was ≥4.The number of cases in the respiratory muscle paralysis， cranial nerve palsy and autonomic symptoms in the AMAN was more than in the AIDP group，but there was no statistical significance. In the sensory nerve involvement， the AIDP was obviously more severe than in the AMAN group. Conclusion　The incidence of the AMAN and the AIDP group is similar in north Chinese children.Compared with AIDP group，the AMAN group has seasonal characteristics and gender differences. Between the two groups， besides sensory nerve involvement ，there was no significant difference in the clinical feature and severity. However， the clinical progress of the AMAN is faster than the AIDP group.