儿童Miller-Fisher综合征和Guillain-Barré综合征的神经电生理对比分析

Neuroelectrophysiology of Miller-Fisher syndrome versus Guillain-Barrésyndrome in children

目的分析儿童Miller-Fisher综合征(Miller-Fisher syndrome,MFS)与Guillain-Barré综合征(Guillain-Barrésyndrome,GBS)的各项神经电生理指标并比较两者的差异,探究两者的神经电生理特征。方法参照2014年多国专家分类组制定的GBS-MFS诊断标准,回顾性收集河南省儿童医院自2013年1月至2020年6月住院确诊为MFS病例33例和GBS病例105例。33例MFS患儿中,男童23例,女童10例,年龄(78.15±43.22)个月;105例GBS患儿中,男童69例,女童36例,年龄(64.73±33.90)个月。收集整理入选患者的神经电生理资料,采用t检验、秩和检验、χ^(2)检验对数据进行统计分析。结果33例MFS患儿中,H反射异常19例(57.6%),感觉神经传导异常14例(42.4%),正常神经电生理9例(27.3%);105例GBS患儿中,运动神经传导异常98例(93.3%),H反射异常92例(87.6%),感觉神经传导异常62例(59.0%),针电极肌电图异常44例(41.9%)。感觉神经异常指标中,MFS患儿感觉神经动作电位(SNAP)波幅降低(33.3%,11/33)发生率最高,GBS患儿SNAP未引出(36.2%,38/105)发生率最高。GBS患儿较MFS患儿SNAP波幅降低、感觉神经传导速度(SCV)减慢(均P<0.05)。结论MFS患儿神经电生理异常主要为H反射异常和感觉神经异常,感觉异常指标以SNAP波幅降低最常见,GBS患儿则表现为运动和感觉神经均异常,且以运动神经异常最显著。MFS与GBS神经电生理有明显差异,认识这些差异可以为MFS、GBS的早期诊断及鉴别诊断提供更多的参考依据。

Objective To analyze nerve electrophysiology indicators of Miller-Fisher syndrome(MFS)versus Guillain-Barrésyndrome(GBS)in children,and to explore the nerve electrophysiology characteristics of both.Methods Referring to the GBS-MFS diagnostic criteria formulated by the multinational expert classification team in 2014,33 cases diagnosed with MFS and 105 cases diagnosed with GBS were retrospectively collected from Henan Children's Hospital from January 2013 to June 2020.In the 33 cases of MFS,there were 23 boys and 10 girls who were(78.15±43.22)months old.In the 105 cases of GBS,there were 69 boys and 36 girls who were(64.73±33.90)months old.The neuroelectrophysiological data of the children were collected and sorted out.The data were statistically analyzed by t,rank sum,andχ^(2) tests.Results Among the 33 children with MFS,19 cases(57.6%)had abnormal H-reflex,14 cases(42.4%)abnormal sensory nerve conduction,and 9 cases(27.3%)normal nerve electrophysiology;among the 105 children with GBS,98 cases(93.3%)had abnormal motor nerve conduction,92 cases(87.6%)abnormal H-reflex,62 cases(59.0%)abnormal sensory nerve conduction,and 44 cases(41.9%)abnormal needle electrode electromyography.Among the indicators of sensory nerve abnormalities,the incidence of decreased amplitude of sensory nerve action potential(SNAP)in the MFS children was the highest(33.3%,11/33),and the incidence of SNAP not extracted in the GBS children was the highest(36.2%,38/105).The GBS children had lower SNAP amplitude and slower sensory conduction velocity(SCV)than the MFS children(both P<0.05).Conclusions The nerve electrophysiology abnormalities in MFS children are mainly H-reflex abnormality and sensory nerve abnormality;the most common indicator of paresthesia is SNAP amplitude reduction.The nerve electrophysiology manifestations in GBS children are motor and sensory nerve abnormalities,and the motor nerve abnormalities are the most significant.There are obvious differences in nerve electrophysiology between children with MFS and GBS.Understanding these differences can provide more references for the early diagnosis and differential diagnosis of MFS and GBS.