91例肌萎缩侧索硬化症的F波和神经传导研究

目的探讨肌萎缩侧索硬化症(ALS)F波和神经传导改变的特点.方法所有患者均采用常规方法测定感觉神经传导速度(SCV)、运动末端潜伏期(distal motor latency,DML)和F波,后者的测定包括潜伏期和/或传导速度及出现率.分析了DML和复合肌肉动作电位(compound muscle action potential,CMAP)波幅、F波出现率与肌力的关系.结果在91例ALS患者中,仅有3例SCV异常;正中神经、尺神经及胫后神经DML延长者分别占16.7%、13.8%、7.1%,CMAP波幅下降者分别占50.0%、44.6%、28.6%;5.0%患者F波传导速度异常,48例患者F波出现率下降,其中19例出现率为0.肌力下降者DML、CMAP波幅及F波出现率改变明显.结论ALS患者可出现DML延长和CMAP波幅降低,二者比较后者的改变更显著;F波传导速度相对正常而出现率下降明显;DML、CMAP波幅及F波出现率的异常与肌力明显相关(P均＜0.01).     

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     

婴儿型脊肌萎缩症的神经电生理特点

目的探讨婴儿型脊肌萎缩症(SMA)的神经电生理特征。方法采用肌电/诱发电位仪对27例SAM患儿进行神经传导速度测定,每例进行至少6块肌肉的EMG检查,分析检查结果。结果婴儿型SMA正中神经复合肌肉动作电位(CMAP)波幅显著下降,尺神经与腓总神经的CMAP波幅明显下降,伴有尺神经运动传导速度的轻度减慢;所记录感觉神经传导未见明显异常。EMG提示神经源性损伤。结论婴儿型SMA典型临床症状为进行性加重的对称性肌肉无力和萎缩,特异性的神经电生理表现为本病的诊断提供重要的依据。     

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。     

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。     

神经电生理检查在肯尼迪病和进行性肌萎缩鉴别诊断中的价值

目的 通过观察神经电生理改变特点,协助鉴别肯尼迪病(KD)和进行性肌萎缩(PMA).方法 收集13例KD和12例PMA患者,对两组患者运动和感觉神经传导、常规肌电图、单纤维肌电图结果进行比较.结果 同侧肢体正中神经(拇短展肌记录)和尺神经(小指展肌记录)远端复合肌肉动作电位(CMAP)波幅比值在KD组和PMA组分别为1.11±0.40、0.57±0.29(t=4.760,P=0.000).6例KD患者感觉神经传导测定显示波幅下降,传导速度正常.PMA患者感觉传导测定均正常.单纤维肌电图检测可见,KD组和PMA组平均颤抖(jitter)分别为(56.3±24.2)、(97.1±35.3)μs(t=2.696,P=0.015),阻滞百分比中位数(M_(50))分别为0、32.5%(Z=3.168,P=0.002).两组肌电图检查均表现为广泛神经源性损害.结论 KD和PMA进行鉴别时,如运动传导显示正中神经CMAP波幅大于尺神经波幅,感觉神经传导波幅下降,单纤维肌电图颤抖相对正常,可以支持KD的诊断.     

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.     

神经型布氏杆菌病周围神经损害的临床与电生理研究

目的研究神经型布氏杆菌病周围神经损害的临床特征,探讨电生理对其的诊断价值。方法对32例神经型布氏杆菌病周围神经损害患者(病例组)和32名性别及年龄与病例组匹配的正常对照组进行神经电生理检查,并对所得检查结果进行统计学分析。结果病例组与对照组在运动末梢潜伏期(distal motor latency,DML)、复合肌肉动作电位(compound motor active potentials,CMAP)波幅、运动神经传导速度(motor nerve conduction velocity,MCV)、感觉神经动作电位潜伏期(sensory nerve action potential latency,SL)、感觉神经动作电位(sensory nerve action potential,SNAP)波幅及感觉神经传导速度(sensory nerve conduction velocity,SCV)方面的比较,差异均有统计学意义(P0.05)。电生理检查提示上下肢周围神经损害,感觉神经及运动神经均受累,其中感觉神经占55.47%,运动神经占16.80%,上肢以正中神经(64条)最多见,下肢以腓肠神经(16条)最多见。四肢运动神经256条中43条传导速度减慢,占16.80%,四肢感觉神经256条中142条传导速度减慢,占55.47%,SCV较MCV改变明显,上肢病变重于下肢。结论神经电生理检查为神经型布氏杆菌病周围神经损害的临床诊断提供了客观依据。     

糖尿病周围神经病病情分级与电生理的相关性

目的探讨糖尿病周围神经病病情分级与电生理的相关性。方法依据糖尿病性周围神经病的诊断标准确定入选对象;依据糖尿病周围神经病病情分级对入选对象进行临床分级;应用丹麦产DANTEC CANTATA型肌电图仪,进行运动神经和感觉神经传导功能检查。结果腓肠神经、正中神经诱发感觉动作电位波幅(SNAP)和腓总神经复合肌肉动作电位波幅(CMAP)随病情分级的升高而明显减低(P<0.05);腓肠神经、正中神经感觉传导速度(SCV)和腓总神经、正中神经运动传导速度(MCV)3级与1、2两级比较显著减慢(P<0.05)。结论神经电生理改变,尤其感觉神经电生理改变,易此作为糖尿病周围神经病情程度评定的指标。     

神经元核内包涵体病的电生理特点及其辅助诊断价值分析

目的总结神经元核内包涵体病(NIID)的电生理特点并探讨电生理检查在其辅助诊断中的价值。方法选择徐州医科大学附属医院神经内科自2020年2月至2022年6月收治的20例经皮肤活检和基因检测确诊的NIID患者(15例有症状, 5例无症状)进行研究, 分析其周围运动/感觉神经传导、针极肌电图、F波、重复电刺激、交感神经皮肤反应(SSR)、震颤分析等电生理检查结果, 并对15例有症状NIID患者(有症状NIID组)与11例年龄、性别相匹配的正常健康者(对照组)的周围神经传导和SSR指标进行比较。结果 (1)15例有症状NIID患者均存在电生理检查结果异常:14例存在周围神经传导异常, 其中运动神经传导速度(MCV)减慢14例, 复合肌肉动作电位(cMAP)波幅降低4例, 感觉神经传导速度(SCV)减慢12例, 感觉神经动作电位(sNAP)波幅降低3例, 总体表现出对称的神经传导速度减慢而波幅相对保留;4例针极肌电图显示为神经源性损害;13例F波潜伏期有不同程度延长;12例出现SSR异常;4例表现出4.0～7.5 Hz的同步性震颤。(2)5例无症状NIID患者中3例存在周围神经传导异常, 其中...     

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     

F-wave amplitude in peripheral nervous system lesions

BACKGROUND AND PURPOSE: F-wave is a late response recorded from muscle elicited by electric impulse conveyed antidromically to alpha-motor neurons of the spinal cord. F-wave latency and frequency are assessed in routine electroneurography providing information of conduction in the proximal segment of the nerves. F-wave amplitude is rarely analyzed, while it could add valuable information on excitability of motor neurons in different disease states. This study was conducted to determine whether F-wave amplitude is indicative of the level of the peripheral nervous system lesion. MATERIAL AND METHODS: EMG recordings of 204 consecutive patients suspected of peripheral nerves system lesion were retrospectively analyzed. Based on the clinical diagnosis three groups were defined: neuropathy (N=100), myopathy (N=33), ALS (N=18), reference (musculoskeletal pain syndromes: N=53). F-wave amplitude and F/CMAP-ratio (CMAP-compound motor action potential) and their relation to parameters of impulse conduction in motor nerve fibers was analyzed. RESULTS: Mean F/CMAP ratio was 11.1% in ALS, 5% in myopathy (p=0.008), 7.1% in neuropathies and 5.6% in the reference group. Giant F-wave (more than 10% of CMAP) was observed in 30% of nerves in ALS, 15% in neuropathy and 10% in myopathy (p=0.036). F-wave amplitude correlated significantly with CMAP amplitude in all groups, while F/CMAP ratio was inversely related to CMAP amplitude in ALS (r=-0.43, p<0.01) and neuropathy (r=-0.37, p<0.0001). F-wave frequency was similar in all groups and correlated with CMAP amplitude. CONCLUSIONS: F-wave amplitude is not indicative of the level of peripheral nervous system lesions. Giant F-wave is observed in neurogenic processes. It reflects an increase of motor unit size in the reinnervation process, but possibly also a change of excitability of motor neuron and its axon.     

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.     

Clinical value of F-wave recordings in traumatic cervical spinal cord injury

F-waves and motor/sensory nerve conduction (NCS) of the median and ulnar nerves were examined in 66 patients with traumatic motoneurone lesion due to acute and chronic cervical spinal cord injury (SCI). The examinations were performed in parallel in chronic tetraplegics once and in acute tetraplegic patients monthly for the first 3 months, after 6 months and 1 year post-trauma. A pathological reduction of the compound muscle action potential (CMAP) (in 10% even a complete loss of the CMAP) was present in about 50% of the patients. The mean CMAP values of tetraplegic patients with either acute or chronic SCI were significantly (P < 0.001) reduced compared to normal subjects. Because sensory nerve conduction in these patients was normal, the reduction of CMAP should be due to damage of intramedullar motoneurones or anterior nerve roots. While in all chronic SCI patients with preserved CMAP F-waves could be elicited, 50% of the acute SCI patients showed a complete loss of F-waves of both nerves during the initial examination due to spinal shock. After 6 months all acute SCI patients with preserved motor potentials regained F-waves. Therefore, the excitability of F-waves is influenced by spinal shock in acute SCI. The mean F-wave latencies (Fmin-response, Fmin-M response) revealed no significant difference between healthy subjects and SCI patients. However, the frequency of F-wave production was related to the severity of the motoneurone lesion. Furthermore, while the F-wave latencies and CMAP values did not change significantly with time after acute SCI, the frequency of F-wave production increased, but remained reduced compared to normal subjects.     

Pathophysiology inferred from electrodiagnostic nerve tests and classification of polyneuropathies. Suggested guidelines.

OBJECTIVE: To present criteria for pathophysiological interpretation of motor and sensory nerve conduction studies and for pathophysiological classification of polyneuropathies suggested by a group of European neurophysiologists. METHODS: Since 1992 seven neurophysiologists from six European countries have collected random samples of their electrodiagnostic examinations for peer review medical audit in the ESTEEM (European Standardized Telematic tool to Evaluate Electrodiagnostic Methods) project. Based on existing criteria in the literature, the experience with a patient material of 572 peer reviewed electrodiagnostic examinations, and productive discussions between the physicians at workshops, the collaboration has produced a set of criteria now routinely used at the centres involved in the project. RESULTS: The first part of the paper considers pathophysiology of individual nerve segments. For interpretation of motor and sensory nerve conduction studies, figures showing change in amplitude versus change in conduction velocity/distal latency and change in F-wave frequency versus change in F-wave latency are presented. The suggested boundaries delimit areas corresponding to normal, axonal, demyelinated, or neuropathic nerve segments. Criteria for motor conduction block in upper and lower extremities are schematically depicted using the parameters CMAP amplitude and CMAP duration. The second part of the paper suggests criteria for classification of polyneuropathies into axonal, demyelinating, or mixed using the above-mentioned criteria. CONCLUSIONS: The suggested criteria are developed during many years of collaboration of different centres and may be useful for standardization in clinical neurophysiology. SIGNIFICANCE: Consistent interpretation of nerve conduction studies is an important step in optimising diagnosis and treatment of nerve disorders.     

Peripheral nerve abnormalities in pediatric patients with spinal muscular atrophy

We examined the specific nerve conduction deficits distinguishing spinal muscular atrophy (SMA) subtypes I and II. Five SMA I patients (age, 0.2–1.1 years) and 10 SMA II patients (age, 1.0–2.8 years) were examined. Patients were compared to age-matched controls for motor and sensory conduction velocity (MCV and SCV) changes, compound muscle and sensory nerve action potential amplitudes (CMAP and SNAP), and F-wave occurrence (FO). Slower MCVs were found in three of five SMA I patients; all five exhibited markedly decreased CMAP amplitudes. Tibial nerve CMAP amplitudes significantly reduced in SMA II patients (p < 0.01). Slower SCVs and decreased SNAP amplitudes were observed in three of five SMA I patients but not in SMA II patients. Although FOs were reduced in both extremities of SMA I patients, the reduction was prominent in the tibial nerve of SMA II patients (p = 0.031). Loss of motor units may be widespread in the early stage of SMA I, while specific to the legs in young SMA II patients. SMA I showed sensory nerve degeneration, especially of large myelinated fibers. SMA II showed no sensory nerve abnormalities.     

Electrophysiological features of Hirayama disease

Introduction: The purpose of this study was to compare the pattern of hand muscle involvement in Hirayama disease (HD) and amyotrophic lateral sclerosis (ALS). Methods: We reviewed findings of upper limb nerve conduction studies of 46 HD patients and 60 ALS patients. The findings from 54 healthy subjects were used for comparison. Results: In HD, the ulnar compound muscle action potential (CMAP) amplitude was more severely reduced than the median one, and the reverse pattern was observed in ALS. The mean ulnar/median (U/M) CMAP amplitude ratio was significantly lower in HD (0.64 ± 0.79) and abnormally higher in ALS (2.15 ± 1.77) compared with normal subjects (0.89 ± 0.23). An abnormally low U/M CMAP amplitude ratio (<0.6) was encountered in 34 patients with HD and in 1 with ALS. A U/M CMAP amplitude ratio ≥4.5 or absent median motor response was found only in ALS. Conclusion: Our findings demonstrate different patterns of hand muscle involvement between these two diseases. Muscle Nerve, 2011