脂质沉积性肌病患者肌电图与肌力和肌酸磷酸激酶的相关性  

The relationship between muscle strength, creatine kinase and electromyography in lipid storage myopathy

作　　者：刘明生[1] 崔丽英[1] 徐丹[1] 管宇宙[1] 李秀丽[1] 陈琳[1] 

机构地区：[1]中国医学科学院北京协和医院神经科,100730

出　　处：《中华神经科杂志》2014年第6期392-395,共4页Chinese Journal of Neurology

摘　　要：目的：分析脂质沉积性肌病患者肌电图与肌力和肌酸磷酸激酶之间的相关性，进一步提高对脂质沉积性肌病的认识。方法在病理确诊的脂质沉积性肌病患者中，收集运动神经传导、感觉神经传导、F波以及同芯针肌电图测定结果。对肌电图改变特点及其与肌力和肌酸磷酸激酶之间的相关性进行分析。结果在30例患者中，肌电图表现为肌源性损害的19例，肌源性损害合并神经性损害5例，单纯神经源性损害2例，未见异常4例。16/123的感觉神经传导存在异常。在16/74块肌肉检测到异常自发电位。肌电图有异常自发电位组和无异常自发电位组的血清肌酸磷酸激酶水平[M（Q25， Q75）]分别为295.5（201.0，4845.8） U/L和482.0（292.8，963.8） U/L （Z＝0.281， P＝0.778）。运动单位电位表现为肌源性损害组和非肌源性损害组的肌酸磷酸激酶水平[ M（ Q25， Q75）]分别为332.0（267.0，1932.0） U/L和502.0（224.5，877.5） U/L （Z＝0.091， P＝0.928）。有异常自发电位组和无异常自发电位组的三角肌英国医学研究委员会（ Medical Research Council ， MRC）评分分别为（4.3±0.7）分、（4.1±0.8）分（t＝0.490， P＝0.628），股四头肌MRC评分分别为（3.9±0.6）分、（3.7±0.6）分（t＝0.725， P＝0.474）。运动单位电位表现为肌源性损害组和非肌源性损害组的三角肌MRC评分分别为（4.1±0.7）分、（4.1±0.9）分（t＝0.101， P＝0.920），股四头肌MRC评分分别为（3.8±0.6）分、（3.7±0.7）分（ t＝0.368，P＝0.716）。结论脂质沉积性肌病肌电图检查可以出现感觉神经传导异常，运动单位电位呈神经源性损害表现或正常，而异常自发电位相对少见；自发电位和运动单位电位时限的改变与肌酶和肌力并不相关。Objective To assess the relationship between muscle strength , creatine kinase （ CK） and electromyography （ EMG） in patients with lipid storage myopathy （ LSM）.Methods Motor and sensory nerve conduction study, F wave, and quantity EMG were performed in 30 patients with LSM.Muscle strength and CK were studied before EMG.The relationship between EMG , muscle strength and CK were analyzed.Results EMG showed myopathic changes in 19 patients,myopathic coexistence with neuropathic changes in five , and neuropathic changes in two.Four patients had normal EMG.Motor nerve conduction studies showed decreased compound muscle action potential amplitude in seven nerves of two patients , while abnormal sensory nerve conduction in 16 nerves of five patients.Quantity EMG was performed in 78 muscles.Thirty-eight muscles of 21 patients had decreased amplitude and short duration motor unit potential （ MUP） , 6 muscles of 2 patients had increased amplitude and long duration MUP , and 30 muscles of 16 patients had normal MUP.Abnormal spontaneous activities （ SA） were detected in 16 muscles.The M（ Q25 , Q75 ） of serum CK was 295.5 （201.0, 4 845.8） U/L in patients with abnormal SA in EMG , and 482.0 （292.8, 963.8） U/L in patients without abnormal SA in EMG （Z=0.281, P=0.778）.Medical Research Council （ MRC） Scale in deltoid muscle was 4.3 ±0.7 in muscles with SA and 4.1 ±0.8 in muscles without SA （t=0.490, P=0.628）, and that in quadriceps femoris was 3.9 ±0.6 for muscles with SA and 3.7 ±＆nbsp;0.6 for muscles without SA （ t =0.725 , P =0.474 ）.MRC Scale in deltoid muscle was 4.1 ±0.7 in muscles with myogenic MUP and 4.1 ±0.9 in muscles without myogenic MUP （ t=0.101, P=0.920）, which in quadriceps femoris was 3.8 ±0.6 for muscles with myogenic MUP and 3.7 ±0.7 for muscles without myogenic MUP （ t=0.368 , P=0.716 ）.Conclusions Neurogenic or normal MUP and abnormal sensory nerve conduction can be presented in some patients with LSM , while SA is not common.The changes in MUP

关 键 词：脂质代谢缺陷 先天性 肌营养不良 肌电描记术 肌力 肌酸激酶 

分 类 号：R746[医药卫生—神经病学与精神病学]