肌萎缩侧索硬化患者205例的神经传导特点分析

目的 分析肌萎缩侧索硬化(ALS)患者的神经传导和F波特点,并探讨其与肌力、病程和首发部位等之间的关系.方法 收集于1997年1月至2008年5月期间我院门诊或病房收治的ALS患者205例,均采用常规肌电图检查,测定其运动神经传导、F波以及感觉神经传导(SCV).结果 在205例ALS患者中,仅有3例SCV异常,正中神经、尺神经及胫后神经末端潜伏期(DML)延长者分别占24.9%(48/193)、15.3%(25/163)、21.2%(7/33),复合肌肉动作电位(CMAP)波幅下降者分别占57.0%(110/193)、49.7%(81/163)、39.4%(13/33);68.9%(122/177)患者F波出现率下降,其中31.1%(55/177)F波出现率为0,肌力下降者DML、CMAP波幅及F波出现率改变明显.肢体起病组正中神经CMAP波幅下降[81.5%(53/65)]和F波异常率[70.9%(44/62)出现率下降,45.1%(28/62)出现率为0]较延髓部起病者[32.4%(11/34);F波38.2%(13/34)出现率下降,14.7%(5/34)出现率为0]更明显,两组比较差异有统计学意义(x2=23.629、9.753、9.029,均P<0.01);DML异常两组间差异无统计学意义.Logistic回归分析显示CMAP波幅的降低与上肢远端肌力、首发部位、病程显著相关,F波出现率的降低与上肢远端肌力、首发部位相关.结论 ALS患者可出现DML延长和CMAP波幅降低(后者改变更显著),F波出现率明显下降而传导速度相对正常;DML、CMAP波幅及F波出现率的异常与肌力明显相关.首发部位为肢体和(或)上肢远端肌力下降者CMAP波幅及F波异常率更明显.

Abstract：

Objective To investigate the F-wave and nerve conduction in patients with amyotrophic lateral sclerosis (ALS) and explore the correlation between these parameters and muscle strength, disease duration and onset site.Methods The data of outpatients and inpatients diagnosed with ALS were collected in Peking Union Medical College Hospital from January 1997 to May 2008.Standard sensory and motor nerve conduction study of the median nerve, ulnar nerve and tibial nerve was performed in 205 patients with ALS.F-wave velocity and frequency was measured in median nerve.Parameters for analyses included sensory conduction velocity and amplitude, distal motor latency (DML), and compound muscle action potential (CMAP) amplitude.Correlation between muscle strength and DML, CMAP amplitude or F-wave frequency were also explored.Results Delayed DML of the median nerve, ulnar nerve and tibial nerve were found in 24.9% (48/193), 15.3% (25/163), 21.2% (7/33) of patients respectively.Decreased CMAP amplitudes were found in 57.0% (110/193), 49.7% (81/163), 39.4% (13/33) of patients respectively.Decreased F-wave frequency of the median nerve was found in 68.9% (122/177) of patients.The abnormality of DML,CMAP amplitude and F-wave frequency of median nerve were increased in weaker muscles.Decreased median nerve CMAP amplitude (81.5% (53/65)) and F-wave abnormality (decreased persistence 70.9%(44/62), absent responses 45.1% (28/62)) in spinal onset groups were significantly higher than those in bulbar onset groups (CMAP 32.4% (11/34); F-wave: decreased persistence 38.2% (13/34), absent responses 14.7% (5/34); x2 = 23.629, 9.753, 9.029,all P <0.01).Compared with the bulbar onset group,the abnormality of DML in spinal onset group was higher, but not reach statistical significance.Logistic regression revealed a strong direct association between decreased CMAP amplitudes and upperextremity muscles strength, disease duration and onset symptom.Abnormality of F-wave frequency was associated with upper-extremity muscles strength and onset symptom.Conclusions Delayed DML and decreased amplitude of CMAP are found in ALS patients.CMAP amplitude is a sensitive parameter related to the severity of ALS.F-wave velocity is relatively normal while F-wave frequency of the median nerve is correlated with muscle strength.Decreasing CMAP amplitude and F-wave frequency are correlated strongly with muscle weakening,disease duration and symptom onset over limbs.     

神经传导速度在肌萎缩侧索硬化中的诊断价值

目的　研究肌萎缩侧索硬化 (ALS)患者中神经传导速度的改变 ,建立量化评定肌萎缩侧索硬化病情轻重的神经生理指数。方法　对 2 1名ALS患者的 3 0条尺神经、3 2条正中神经及 2 4名健康对照组的 3 8条尺神经、40条正中神经进行神经运动传导速度 (MCV)及感觉传导速度 (SCV)和F波的检测。两组间数据进行统计学分析。结果　ALS组正中神经、尺神经运动传导速度的远端潜伏期 (DML)、肌肉动作电位 (CAMP波幅及面积、F波的出现率 )较对照组有显著性差异。而两组MCV、SCV、F波的潜伏期差异无显著性。ALS组中 1 0名小指展肌的肌力与 (CAMP波幅 /DML×F出现率 )的数值有显著的相关性 (r=0 89,P <0 0 1 )。结论　 (CMAP波幅 /DML×F波的出现率 )是一种有效的客观的电生理指数 ,可对ALS病情进行量化评估     

肌萎缩侧索硬化患者神经传导分析

目的分析肌萎缩侧索硬化(ALS)患者的神经传导特点,并探讨神经传导与不同起病部位之间的关系。方法收集于2012年1月至2014年12月期间我院门诊或病房收治的ALS患者47例和体检的健康对照组40例,均测定其运动及感觉神经传导、F波。结果ALS患者运动神经传导检测表现为潜伏期(DML)延长,复合肌肉动作电位(CMAP)波幅下降,传导速度(MCV)减慢,其中以CMAP波幅下降为主。可见"分裂手"和"分裂足"现象。部分病人的症状、体征或电生理出现感觉神经受累证据。部分病人的F波出波率异常。结论诊断ALS需对临床和神经电生理进行综合分析,不能仅凭其中一项而轻易排除诊断。临床医生,特别是基层医院医生,要全面细致地完善辅助检查结果,减少漏诊和误诊的发生。     

肌萎缩侧索硬化患者膈神经传导的电生理分析

目的 通过分析肌萎缩侧索硬化(Amyotrophic Lateral Sclerosis,ALS)患者膈神经传导检测,并结合其它神经电生理资料,为该病提供更深入的认识,进一步指导临床诊疗。方法 研究范围为武汉大学人民医院2014年1月-2021年12月就诊的ALS患者共88例,收集患者的一般资料、主要症状及体征、肌萎缩侧索硬化改良量表(ALSFRS-R)评分、运动神经传导检测中的复合肌肉动作电位(CMAP)波幅和远端运动潜伏期(DML)等指标。结果(1)运动神经传导检测中CMAP波幅降低192条(43.6%),膈神经波幅异常率为35.2%; 远端潜伏期延长116条(26.4%),膈神经DML异常率为77.3%;(2)膈神经DML在性别方面存在明显差异(P<0.01);(3)ALSFRS-R评分与膈神经、尺神经、正中神经、腓总神经、胫神经的CMAP波幅呈正相关(r=0.393,P<0.01; r=0.375,P<0.01; r=0.413,P<0.01; r=0.251,P<0.05; r=0.442,P<0.01);(4)膈神经DML及CMAP波幅在起病部位方面存在明显差异(P<0.05; P<0.05);(5)膈神经DML在判断病情中度和轻度之间的最佳界点为9.095 ms,敏感性为85.7%,特异性为80.2%。结论 ALS患者的运动神经传导可表现异常,CMAP波幅下降占比较大,但膈神经中潜伏期延长比CMAP波幅降低更多见。膈神经传导检测存在一定程度的性别差异。行运动神经传导检测时多条神经CMAP波幅变化可反映ALS患者病情严重程度。膈神经潜伏期变化可更敏感地反映ALS的病情严重程度,以期指导临床诊断与治疗。     

神经传导检测在Bell麻痹预后判断中的价值

目的:探讨神经传导检测在Bell麻痹预后判断中的价值.方法:对发病10～21 d的56例Bell麻痹患者进行神经传导检测和F波检查,以随访3个月时患者的House-Brackmann分级结果来判断神经传导检测的价值.结果:预后好组和预后差组患侧面神经复合肌动作电位(CMAP)波幅分别为1.15(0.90,1.60)mV和0.20(0.13,0.40)mV,远端运动潜伏期(DML)分别为2.70(2.40,3.00)ms和3.00(2.85,3.68)ms,波幅下降比分别为(31.14±18.47)%和(79.03±13.14)%,F波出现率分别为25.00(15.00,30.00)%和5.00(0.00,15.00)%,两组比较差异有显著意义.结论:虽然神经传导检测和F波是评价面神经功能的敏感方法,但是CMAP波幅、DML并不适合作为Bell麻痹的预后判断指标,F波出现率也可能不可靠,患侧波幅下降比仍然是判断预后的较好指标.     

肌萎缩侧索硬化患者早期选择性损害快速传导运动神经元的电生理研究

目的分析早期肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)患者正中神经F波最小潜伏期(F wave minimal latency,Fmin)和运动末端潜伏期(distal motor latency,DML)改变及其与复合肌肉动作电位(compound muscle action potentials,CMAP)负波波幅和负波面积的相关性,寻找ALS早期选择性损害快速传导运动神经元的证据。方法纳入确诊和很可能的ALS(改良EI Escorial标准)患者42例,选择性别构成、年龄及身高与之匹配的健康自愿受试者46名为对照组。采用电生理检测手段检测所有对象正中神经DML、Fmin、CMAP负波波幅和负波面积等指标,比较两组间以上指标的差异,并通过Pearson相关性分析方法分析ALS患者DML、Fmin、Fmax与CMAP负波波幅、负波面积的相关性。结果与对照组比较,ASL组正中神经Fmin延长(P0. 01),DML虽有延长趋势但差异无统计学意义(P0. 05),正中神经负波波幅及负波面积较对照组下降(P0. 01)。ALS患者正中神经CMAP负波波幅、负波面积均与DML(r=-0. 433,P=0. 005;r=-0. 320,P=0.041),及Fmin(r=-0. 669,P0. 001;r=-0. 597,P0. 001)呈线性负相关,与Fmax(r=-0. 283,P=0.072;r=-0.220,P=0. 166)无相关性。结论早期ALS患者Fmin与CMAP波幅、负波面积呈负相关,提示快传导的大运动神经元在ALS早期优先受累。     

正中神经和尺神经分段刺激在慢性炎性脱髓鞘性多发性神经病中的电生理研究

目的 探讨运动神经传导速度(MCV)、复合肌肉动作电位(CMAP)与肌力减退的关系和传导阻滞(CB)在慢性炎性脱髓鞘性多发性神经病(chronic inflammatory demyelinating polyradiculoneuritis,CIDP)中的表现特点.方法 30例CIDP患者在进行常规MCV、远端潜伏期(DML)、F波、感觉神经传导速度(SCV)、肌电图(EMG)测定的基础上,在正中神经采用由远到近的“腕-肘-腋-Erb's点”4点3段刺激,尺神经采用由远到近的“腕-肘下-肘上-腋-Erb's点”5点4段刺激,记录各段刺激后CMAP各参数及MCV的变化.结果 CMAP波幅衰减、面积衰减、时程增加以及MCV减慢与临床肌力减退无相关性,dCMAP波幅与上肢远端肌力呈正相关;患者中80.00％在正中神经、73.33％在尺神经发现了1个或多个节段的CB,且出现节段无明显选择性.结论 dCMAP波幅降低与CIDP患者肌力减退有相关性.在CIDP中CB出现率高,且较为弥散地在各节段中出现.     

45例正中神经腕管卡压神经电生理检测与临床分析

目的探讨正中神经腕管卡压(CTS)神经电生理检测价值。方法对临床的症状及体征符合CTS的45例患者行正中神经运动神经的传导速度与尺神经运动神经的传导速度检测;桡神经与正中神经拇指-腕感觉潜伏期时差值;正中神经与尺神经无名指-腕感觉潜伏期时差值;双侧正中神经F波的检测;拇短展肌、小指展肌的肌电图检测。结果 45例患者中63只异常,双侧病变18例,单侧病变27例,正中神经运动末端潜伏期延长或(及)传导速度减慢异常率31.5%,波幅减低异常率28.3%;正中神经拇指-腕感觉神经潜伏期延长异常率71.5%;合并波幅减低者异常率占79.3%;正中神经环指-腕感觉神经传导潜伏期延长异常率81.6%,合并波幅减低异常率89.4%;正中神经F波异常率33.6%;拇短展肌呈神经源性改变异常率20.1%。结论神经电生理的常规检测联合运用感觉神经潜伏期时差值法对CTS有更敏感、更精确的诊断价值。     

视神经脊髓炎谱系疾病周围神经病变的相关神经电生理研究

目的探讨视神经脊髓炎谱系疾病(NMOSD)周围神经病变的神经电生理改变。方法对15例NMOSD患者(NMOSD组)及20名健康对照者(正常对照组)进行EMG检查。结果 NMOSD组中,10例(66.7%)患者的EMG检查有异常改变,主要表现为运动或感觉神经传导速度减慢,波幅减低,F波出现率下降和H反射的潜伏期延长。与正常对照组比较,NMOSD组胫神经及腓总神经运动神经传导速度显著降低(均P0.01),正中神经远/近段潜伏期、胫神经远端潜伏期显著升高(均P0.05),胫神经、腓总神经运动神经远/近端波幅显著降低(均P0.01)。与正常对照组比较,NMOSD组尺神经、正中神经、腓肠神经感觉神经传导速度显著减慢(均P0.01),腓肠神经感觉神经潜伏期显著升高(P0.01),正中神经、腓肠神经感觉神经波幅显著降低(均P0.01)。NMOSD组H反射潜伏期(26.22±10.10)与正常对照组(24.36±0.63)差异无统计学意义(P0.05)。结论 NMOSD患者可以合并周围神经系统受损。EMG检查主要表现为运动或感觉神经传导速度减慢,波幅减低,F波出现率下降和H反射的潜伏期延长。     

早期糖尿病周围神经病患者的EMG改变及其与血清脂蛋白相关磷脂酶A2水平的关系

目的探讨早期糖尿病周围神经病(DPN)患者的EMG改变及其与血清脂蛋白相关磷脂酶A2(Lp-PLA2)水平的关系。方法对90例早期DPN患者行EMG检查,测定内容包括双侧神经运动传导速度(MCV)、复合肌肉动作电位波幅(CMAP)、感觉传导速度(SCV)、感觉神经动作电位波幅(SNAP)及胫神经H波和尺神经f波,分析其与患者血清Lp-PLA2水平的关系。结果 90例早期DPN患者EMG检查总异常率为90.0%。早期DPN患者下肢运动神经异常率(68.6%)显著高于上肢异常率(24.5%)(χ~2=123.863,P=0.000)。早期DPN患者下肢感觉神经异常率(87.4%)显著高于上肢异常率(54.7%)(χ~2=82.712,P=0.000)。早期DPN患者感觉神经总异常率(71.1%)显著高于运动神经总异常率(46.5%)(χ~2=78.978,P=0.000)。DPN患者胫神经H反射总异常65例(72.2%),尺神经f波总异常14例(15.6%)。根据血清LpPLA2水平,90例早期DPN患者分为低水平组(36例)、中水平组(25例)和高水平组(29例)。与低水平组比较,中水平组及高水平组MCV、SCV异常率及CMAP、SNAP下降率均明显降低,且高水平组MCV、SCV异常率及CMAP、SNAP下降率均明显低于中水平组(均P0.05)。Spearman秩相关分析结果显示,血清Lp-PLA2水平与MCV、SCV异常率及CMAP、SNAP下降率均呈负相关(r=-0.741,r=-0.773,r=-0.652,r=-0.611;均P0.05)。结论 EMG检查对DPN早期诊断有重要价值,维持血清Lp-PLA2水平稳定对早期DPN的治疗可能有重要意义。     

Nerve conduction studies in amyotrophic lateral sclerosis

We studied 137 ulnar nerves and abductor digiti minimi (ADM) muscles in 70 patients with amyotrophic lateral sclerosis (ALS), and correlated the results with ADM strength graded on the Medical Research Council (MRC) scale, to address the potential value of a standardized neurophysiological assessment of this nerve-muscle system. The ulnar nerves of 35 normal subjects matched for age, gender, and height served as controls. Reduced compound muscle action potential (CMAP) amplitude and area in the ADM muscle recordings correlated strongly with weakness. Distal motor latency, proximal conduction time, and F-wave frequency were abnormal with minimally detectable weakness. In weaker ADM muscles, conduction velocities and F-wave latencies were also abnormal. Conduction block was never observed and sensory potentials were normal. An "ALS neurophysiological index" was derived from these ulnar nerve studies and consisted of the expression: (CMAP amplitude/DML) x F frequency -, where F frequency was expressed as the number of F responses recorded in 20 trials. This index was strongly correlated with ADM weakness (r = 0.74, P < 0.001). Neurophysiological studies restricted to a single nerve-muscle system, the ulnar nerve/ADM, appear potentially useful in objectively assessing change in ALS.     

F-wave latency,the most sensitive nerve conduction parameter in patients with diabetes mellitus

In this study we examined the diagnostic sensitivity of minimal F-wave latency, F-wave persistence, motor nerve conduction velocity (MCV), and amplitude of the compound motor action potential (CMAP) of the median, ulnar, tibial, and peroneal nerves, and of sensory conduction velocity (SCV) and sensory nerve action potential (SNAP) amplitude of the sural nerve in 82 diabetic patients. For the median, ulnar, and tibial nerves the Z scores of the minimal F-wave latency were significantly larger than those of the MCV, and for all four motor nerves the Z scores of the minimal F-wave latency were significantly larger than those of the amplitude of the CMAP. The Z scores of the peroneal minimal F-wave latency exceeded those of peroneal MCV, sural SCV, and sural SNAP. F-wave persistence did not differ significantly from the reference values. In conclusion, minimal F-wave latency is the most sensitive measure for detection of nerve pathology and should be considered in electrophysiological studies of diabetic patients. © 1997 John Wiley & Sons, Inc. Muscle Nerve 20: 1296–1302, 1997     

Peripheral motor and sensory nerve conduction studies in normal infants and children.

OBJECTIVE: There are few data on electrophysiological data of motor and sensory fibres during nerve maturation. The aim of this study is to investigate the evolution of nerve conduction in the upper and lower limbs during the first years of life. METHODS: The study comprised 92 normal infants and children aged from 1 week to 6 years. Using surface electrodes, the investigation included the following data: (1) motor conduction velocity (MCV), corrected distal motor latency (DML) to a standard distance, and F-waves of the median, ulnar, peroneal and tibial nerves; (2) sensory conduction velocity (SCV) of the median and tibial nerves; and (3) amplitude and morphology of the muscle and sensory action potentials. RESULTS: Maximal MCV and SCV in the neonatal period was about half of adults; there was a steep conduction increase during the first year of life, adult values being reached around age 4. In the neonatal period corrected DML was greater than in adults with a further decrease during the first year. F-wave latencies also decreased during the first year with increase at the end of the study. CONCLUSIONS: This study corroborates the fact that 'maturation' of MCV and SCV occurs during the first 5 years of life, especially in the former. Evolution of DML is accounted for using correction. F-wave latency changes are explained both by an increase in MCV, and extremity growth.     

Interrelationship among nerve conduction velocity, amplitudes of compound muscle and compound nerve action potentials in diabetic neuropathy]

In order to clarify the relationship among amplitudes of compound nerve action potential (CNAP), compound muscle action potential (CMAP) and nerve conduction velocity parameters, data of nerve conduction studies were analyzed in 102 patients with diabetes mellitus. In motor conduction studies CMAP amplitudes after stimulations at the distal nerve trunk, and the polyneuropathy index (PNI), a mean percentage of normal for 12 indices from 4 nerves concerning to the velocity or long distance latency, were evaluated. CNAP was recorded in the median and ulnar nerves from an intrafascicularly inserted microelectrode at the elbow after wrist stimulation. CMAP amplitudes were high in the median and ulnar nerves, and were reduced in the tibial and peroneal nerves. A close relationship was found between PNI and CNAP amplitudes. Among CMAP amplitude parameters tibial nerve, not median or ulnar nerves, had a good correlation with PNI and CNAP amplitude. Along with the progression of diabetic neuropathy, neuropathic signs or symptoms become conspicuous, and nerve conduction velocity drops as is expressed by the PNI level, which reflects the change in nerve conduction velocity in the upper and lower limbs. At the same time CNAP amplitude or CMAP amplitude in the tibial nerve decreases, but in nerves of the upper limb CMAP amplitude doesn't always decrease. So, tibial nerve is best among CMAP amplitude parameters in evaluating the degree of diabetic neuropathy. It is necessary to judge the degree of diabetic neuropathy after due consideration of these facts.     

Length-dependent weakness and electrophysiological signs of secondary axonal loss in chronic inflammatory demyelinating polyradiculoneuropathy

In chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) the pathophysiology underlying permanent muscle weakness and sensory loss was studied in 22 stabilized long-term CIDP patients clinically characterized using isokinetic dynamometry, quantitative sensory testing, and neuropathy scores. Conduction velocity (CV), distal latency (DLAT), minimal F-wave latency (FLAT), compound muscle action potential (CMAP), and amplitude decay between distal and proximal stimulation sites were determined in the median, ulnar, peroneal, and tibial motor nerves and sensory CV and nerve action potentials in the median and sural nerves. Amplitude of CMAP and the DLAT were related to quantitative muscle strength, whereas CV, FLAT, amplitude decay, and dispersion were not consistently related to strength. Furthermore, CMAP and muscle strength were significantly more reduced distally than proximally. In conclusion, the electrophysiological signs of axonal loss and the associated length-dependent muscle weakness suggest secondary axonal loss in addition to primary demyelination in CIDP.     

Correlation between distal motor latency and compound muscle action potential in amyotrophic lateral sclerosis

OBJECTIVES: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder, characterized by a selective progressive degeneration of the motor system. Electromyography is essential for the diagnosis of ALS. The measurement of motor conduction of peripheral nerves is of major importance to recognize other possible causes of progressive muscle wasting. However, there are also pathologic changes in nerve conduction studies in ALS patients. METHODS: In this study we analysed the values of distal motor latency (DML), compound muscle action potential (CMAP) and motor nerve conduction velocity (MNCV) in 95 patients with definite ALS. RESULTS: We found slight slowing of MNCV and moderate to strong reduction of CMAP and a prolongation of DML. We found no significant correlation between MNCV and CMAP. DISCUSSION: The main finding of the present work was the negative correlation between DML and CMAP. It is interpreted as a very distal axonal damage as the main reason for prolongation of DML in ALS patients.     

Peripheral neuropathy in multiple sclerosis: a clinical and electrophysiologic study

Peripheral nerve abnormalities are uncommon in multiple sclerosis (MS). When present, they are usually attributed to factors associated with advanced disease, such as malnutrition or cytotoxic drugs. We prospectively evaluated 22 mildly disabled MS patients with sensory complaints for evidence of neuropathy using the Neuropathy Symptom Score (NSS), clinical examination, and electrophysiologic studies of peripheral nerves. Distal latency, F-wave response, and nerve conduction velocity (NCV) and amplitude in the ulnar, median, tibial, peroneal and sural nerves were examined. Neuropathy was recorded if electrophysiologic abnormalities were detected in at least two peripheral nerves in the same patient. The most frequent electrophysiologic abnormalities noted were prolonged F-wave response and low motor amplitude in the peroneal nerve, slow sensory conduction velocities of the ulnar and sural nerves, and prolonged distal latencies in the sensory ulnar and sural nerves. Electrophysiologic abnormalities were found in 33 of 244 nerves examined (14.7%) and occurred in 10 patients (45.5%). Neuropathic symptoms were mild and did not correlate with electrophysiologic abnormalities. Age, disease duration, disease course and neurologic disability as evaluated by the Kurtzke Expanded Disability Status Scale, were not associated with the presence of neuropathy. Our findings indicate a high frequency of sensory-motor neuropathy in a selected group of MS patients.