运动神经传导阻滞与儿童吉兰-巴雷综合征不同亚型间的关系

Association of motor nerve conduction block with different subtypes of childhood Guillain-Barré syndrome

目的探讨运动神经传导阻滞(CB)与儿童吉兰-巴雷综合征(GBS)不同亚型间的关系。方法回顾性分析50例GBS患儿的临床资料和神经电生理资料,根据神经电生理结果分为2型:急性炎症性脱髓鞘性多发性神经根神经病(AIDP,n=29)和急性运动轴索型神经病(AMAN,n=21)。根据有无运动神经CB分为伴有运动神经CB的AMAN(n=10)、不伴有运动神经CB的AMAN(n=11)、伴有运动神经CB的AIDP(n=19)和不伴有运动神经CB的AIDP(n=10)。比较各组间患儿起病年龄、性别、疾病高峰期休斯功能分级量表(HFGS)评分、短期预后(起病1个月后HFGS评分)。结果AMAN中,运动神经CB均为可逆性。伴有运动神经CB的AMAN的起病1个月后HFGS评分低于不伴有运动神经CB的AMAN(P<0.05),伴有运动神经CB的AIDP的起病1个月后HFGS评分高于伴有运动神经CB的AMAN(P<0.05)。结论伴有可逆性运动神经CB的AMAN提示神经纤维病变轻微,短期预后较不伴有运动神经CB的AMAN和AIDP恢复快。

Objective To study the association of motor nerve conduction block(CB)with different subtypes of childhood Guillain-Barrésyndrome(GBS).Methods A retrospective analysis was performed on the clinical and nerve electrophysiological data of 50 children with GBS.According to the results of nerve electrophysiology,the children were divided into an acute inflammatory demyelinating polyneuropathy(AIDP)group with 29 children and an acute motor axonal neuropathy(AMAN)group with 21 children.According to the presence or absence of motor nerve CB,the children with AMAN or AIDP were further divided into subgroups:group AMAN with or without motor nerve CB(n=10 and 11 respectively)and group AIDP with or without motor nerve CB group(n=19 and 10 respectively).The subgroups were compared in terms of age of onset,sex,Hughes Functional Grading Scale(HFGS)at nadir for the most severe involvement of motor function,and short-term prognosis based on HFGS score at 1 month after disease onset.Results Motor nerve CB was reversible in children with AMAN.AMAN children with motor nerve CB had a significantly lower HFGS score than those without motor nerve CB at 1 month after onset(P<0.05).AIDP children with motor nerve CB had a significantly higher HFGS score than those with motor nerve CB at 1 month after onset(P<0.05).Conclusions AMAN with reversible motor nerve CB suggests mild nerve fiber lesion and has better recovery than AMAN and AIDP without motor nerve CB in short term.