尺神经复合肌肉动作电位改变在吉兰-巴雷综合征和慢性炎性脱髓鞘性周围神经病中的特点

目的：探讨节段和短段刺激尺神经后不同节段复合肌肉动作电位（CMAP）负波各参数变化及传导阻滞在吉兰-巴雷综合征（GBS）和慢性炎性脱髓鞘性周围神经病（CIDP）中的意义。方法：20例GBS和12例CIDP患者行尺神经5点分段刺激（腕、肘下、肘上、腋和Erb＇s点）和短段刺激，记录CMAP负波波幅、时程和面积的变化，分析各参数与临床肌力的相关性。结果：在GBS中，尺神经CMAP负波的时程、波幅和面积的变化在Erb’s点到腕部的各节段中差异很大；波幅与面积的衰减与临床肌力呈高度相关（r=-0.905和-0.907）；传导阻滞多见于近端和肘部，时程离散不明显。在CIDP中，各节段中的参数变化差异不大；远端波幅与临床肌力相关（r=0.586）；传导阻滞在各节段均可出现，常伴明显的时程延长。结论：GBS和CIDP中尺神经CMAP负波的波幅、面积和时程3个参数，可从电生理角度帮助我们认识脱髓鞘疾病的特点。     

Activity-dependent conduction block in multifocal motor neuropathy

Previous studies suggested that activity-dependent conduction block (CB) contributes to weakness in multifocal motor neuropathy (MMN). Obtaining more robust evidence for activity-dependent CB is important because it may be a novel target for treatment strategies. We performed nerve conduction studies in 22 nerve segments of 19 MMN patients, before and immediately after 60 seconds of maximal voluntary contraction (MVC) of the relevant muscle. We employed supramaximal electrical stimulation, excluded nerves with marked axonal loss, and adopted criteria for activity-dependent CB. Per segment, the segmental area ratio [area proximal compound muscle action potential (CMAP)/area distal CMAP] was calculated and, per nerve, total area ratio (area CMAP at Erb's point/area distal CMAP) was obtained. MVC induced no changes in mean area ratios and induced no activity-dependent CB. In segments with CB before MVC, the MVC induced increased duration prolongation. In MMN, MVC induced temporal dispersion but no activity-dependent CB.     

Prevalence of Martin-Gruber anastomosis on motor nerve conduction studies]

Prevalence of median to ulnar anastomosis in the forearm(Martin-Gruber anastomosis; MGA) to the first dorsal interosseous(FDI), abductor digiti quinti (ADQ) and adductor pollicis(AP) was investigated. Subjects contained 106 patients with normal nerve conduction or patients with various neuropathies. Recording electrodes were placed on the motor point of FDI, ADQ and AP. Supramaximal stimulations were given to the median and ulnar nerves at the wrist or above the elbow. The diagnosis of MGA was made by the following criteria; amplitude of compound muscle action potential(CMAP) increased after elbow stimulation as compared with the wrist stimulation in median nerve conduction studies. The corresponding decrease in CMAP amplitude was found after above elbow stimulation as compared with the wrist stimulation in ulnar nerve conduction studies. No MGA was found in 80(75%) out of 106 patients. MGA to FDI was found in all 26 patients who had MGA. MGA to ADQ and AP was found in 11% and 10% of the patients, respectively. Only 8 out of 26 patients had MGA to all 3 muscles. In the presence of MGA median motor nerve conduction studies demonstrate larger CMAP, with a small initial positivity, after elbow stimulation than after wrist stimulation. And moreover, ulnar motor nerve conduction studies reveal a conduction block-like finding in the forearm. In this study MGA was found in 25% of the patient to FDI, in 11% to ADQ and in 10% to AP. Although a very small MGA might be overlooked in our method, such a small MGA doesn't mislead us into erroneous interpretation of motor nerve conduction studies.     

Marinacci communication: an electrophysiological study

OBJECTIVE: Prevalence of Martin-Gruber anastomosis, an anomalous median-to-ulnar forearm communication, is well reported in literature while Marinacci communication, the reverse of Martin-Gruber with a forearm ulnar-to-median communication is under-recognized. We systematically evaluated the presence of Marinacci communication in a series of patients referred for electrophysiological studies. METHODS: One hundred consecutive patients referred to the electrophysiological laboratory for various diagnoses were studied using standard techniques for motor, sensory and f wave studies. RESULTS: Of the 100 patients (200 arms) studied, electrophysiological features of Marinacci communication were observed in 4 patients (7 arms). Median stimulation with recording over abductor pollicis brevis (APB) revealed a pseudo-conduction block over the forearm segment while on ulnar stimulation and recording over abductor digiti minimi (ADM), the amplitude of the compound muscle action potential (CMAP) obtained on proximal stimulation was higher than that obtained on distal stimulation. Ulnar stimulation at the elbow but not at the wrist revealed CMAP from APB without initial positivity. Its amplitude was 50% of the amplitude obtained on median stimulation at the wrist. CONCLUSIONS: Marinacci communication is not uncommon in the general population. A pseudo-conduction block on median stimulation and higher CMAP amplitude on proximal than distal stimulation provide valuable clues to its recognition.     

Amplitude, area and duration of the compound muscle action potential change in different ways over the length of the ulnar nerve.

OBJECTIVE: To study physiological changes of the compound muscle action potential (CMAP) obtained from stimulation at different sites over the full length of a motor nerve and to study possible effects of anthropometrical factors. METHODS: Multicentre study of ulnar motor nerve conduction in five segments to Erb's point performed bilaterally on 100 healthy subjects aged 17-83 years. RESULTS: CMAP amplitude decreased linearly with conduction distance (0.31%/cm) from wrist to Erb's point. CMAP area decreased with the square of conduction distance. Decrease in area was smaller than decrease in amplitude especially distally. CMAP duration increased linearly (0.17%/cm). Amplitude decay correlated with age, height and BMI and dispersion correlated with age and height. There were no correlations between area decay and anthropometrical factors. There was no significant inter-examiner variation. CONCLUSIONS: Area decay may be preferred to amplitude decay in the evaluation of conduction block over short segments due to smaller physiological changes and independence of anthropometrical factors. The absence of inter-examiner variation indicates that the results are robust and may be used by other laboratories. SIGNIFICANCE: This study provides knowledge of physiological changes of CMAP parameters that may be of importance in the evaluation of nerve pathology, in particular conduction block.     

Activity-dependent conduction block in chronic inflammatory demyelinating polyneuropathy

Previous studies suggested that activity-dependent conduction block (CB) contributes to weakness in chronic inflammatory demyelinating polyneuropathy (CIDP). These studies, however, investigated only one nerve segment per patient, employed cervical magnetic stimulation which may be submaximal, included nerves with extremely low compound muscle action potentials (CMAPs) which precludes assessment of CB, and lacked predefined criteria for activity-dependent CB. Obtaining more robust evidence for activity-dependent CB is important because it may be treated pharmacologically. We investigated 22 nerve segments in each of 18 CIDP patients, employed supramaximal electrical stimulation, excluded nerves with markedly reduced CMAPs, and adopted criteria for activity-dependent CB. Each nerve was tested before and immediately after 60 s of maximal voluntary contraction (MVC) of the relevant muscle. Per nerve segment we calculated segmental area ratio: (area proximal CMAP)/(area distal CMAP). Per nerve we calculated total area ratio: (area CMAP evoked at Erb's point)/(area most distally evoked CMAP). MVC induced no change in mean area ratios and no activity-dependent CB according to our criteria, except for one segment. MVC induced increases in distal and proximal CMAP area and duration. In segments with demyelinative slowing, MVC induced an increase in CMAP duration prolongation. Thus, in CIDP, muscle activity induces virtually no CB, but it may increase temporal dispersion of nerve action potentials.     

Electrophysiological measurement at Erb’s point during the early stage of Guillain-Barré syndrome

This study aimed to find the electrophysiological significance of proximal nerve stimulation at Erb’s point during the early stage of Guillain-Barré syndrome (GBS). Twenty-one healthy volunteers and 13 patients within the first week of GBS were studied. Latency and amplitude at wrist, elbow and Erb’s point, and F waves were calculated after compound muscle action potentials (CMAP) were obtained at the median and ulnar nerve. There were statistically significant differences between groups for CMAP latency and amplitude at Erb’s point for the median (p = 0.005 and 0.001, respectively) and ulnar nerves (p = 0.007 and 0.007, respectively). Latency or amplitude of CMAP after Erb’s point stimulation was abnormal in 77% of patients while F wave latency was abnormal in only 46% of patients. Conduction block was observed in 62% of patients. Abnormal parameters at Erb’s point were the only abnormality in four patients at the first electrophysiological examination. We conclude that electrophysiological examination at Erb’s point is a simple and non-invasive method that can be used in the early stage of GBS, especially for patients who exhibit normal F waves and nerve conduction studies at distal nerves.     

Somatosensory evoked potentials from musculocutaneous nerve in the diagnosis of brachial plexus injuries

A technique for recording somatosensory evoked potentials using stimulation of musculocutaneous sensory nerve fibers proximal to the wrist has been used in 10 normal subjects and in 8 patients with traumatic lesions of proximal parts of the brachial plexus. The technique gave satisfactory evoked potentials in all normal subjects and provided useful information in patients with avulsion of the 5th and 6th cervical roots. The results were similar to those obtained by radial nerve stimulation. The results in 3 patients with upper trunk injuries and in 1 patient with avulsion of the 5th cervical root were unhelpful. In 2 patients with multiple cervical root avulsions the evoked potentials from cervical cord and contralateral scalp were absent and were attenuated at Erb's point. This is the first report where musculocutaneous nerve evoked potentials have been applied to a group of patients.     

Analysis of segmental motor conduction in the median and the ulnar nerves: comparison between normal and diabetic individuals]

We investigated characteristics of segmental motor conduction in the median and the ulnar nerves. Subjects were 55 individuals with normal conduction of the upper extremity and 71 patients with diabetes mellitus. Mean polyneuropathy index (PNI), which was determined as a mean percentage of the normal for 6 indices concerning to the conduction velocity in the upper limb, was 99.0% in the normal group and 85.6 % in the diabetic group on the mean. In the normal group distal latency was longer in the median nerve than in the ulnar nerve, and the conduction time between Erb's point and the wrist was longer in the ulnar nerve than the median nerve both in men and women. In the diabetic group these differences were accentuated; that means the distal latency was relatively more prolonged in the median nerve and the conduction time between Erb's point and the wrist was much longer in the ulnar nerve. Prolonged distal latency in the median nerve of women and conduction delay between Erb's point and the wrist in the ulnar nerve of men altogether resulted in the gender difference in the median minus ulnar F-wave latency after wrist stimulation in the diabetic group. Carpal tunnel segment of the median nerve and the elbow segment of the ulnar nerve are known to be common entrapment sites. This phenomenon of accentuated conduction delay in these common entrapment sites might be considered as a sort of 'double crush syndrome'.     

Motor nerve conduction study in cauda equina with high-voltage electrical stimulation in multifocal motor neuropathy and amyotrophic lateral sclerosis

In this study we aim to establish a motor nerve conduction study (NCS) for the cauda equina and examine its usefulness in multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS). NCS of the tibial nerve proximal to the knee was performed with an optimized high-voltage electrical stimulation (HV-ES) method in 21 normal subjects, 5 with MMN, and 11 with ALS. HV-ES, but not magnetic stimulation, could supramaximally stimulate the cauda equina. Cauda equina motor conduction time determined by HV-ES, but not that with F-waves, correlated well with cauda equina length on magnetic resonance imaging. HV-ES revealed proximal lesions in 4 MMN patients but in none of the ALS patients. Importantly, 1 patient with "MMN without conduction block (CB)" had a CB in the cauda equina. Cauda equina motor conduction is better evaluated by HV-ES than with F-wave study or magnetic stimulation. HV-ES can help to distinguish MMN and "MMN without CB" from ALS.     

Multifocal motor neuropathy mimicking motor neuron disease: nine cases.

Multifocal motor neuropathy with persistent conduction block (MMN) is a rare clinical entity, mimicking motor neuron disease (MND). In order to research which are the most frequent nerves and segments where conduction block (CB) can be identified, we reviewed the clinical and neurophysiological data of nine patients with MMN who were studied and followed by the authors. Weakness and muscle atrophy of the dominant hand was the most frequent presentation. Lower limbs were involved later in the disease evolution. The ulnar and median nerves were the most affected nerves. They had conduction blocks mostly at the forearm and at Erb's point-elbow (or above elbow) segments. Both common peroneal and tibial nerves were frequently affected at their distal segments, but proximal segments were also probably involved. The presence of anti-GM1 antibodies was variable, and their determination was not essential for the diagnosis of MMN. Eight patients given IV immunoglobulin therapy had no disease progression. One patient was responsive to corticosteroids. The CB identification in our patients allowed us to clearly distinguish MMN from MND. The good prognosis and need for management with IV immunoglobulin, support the crucial role of a careful neurophysiological study to diagnose this clinical entity.     

Criteria for early detection of conduction block in multifocal motor neuropathy (MMN): a study based on control populations and follow-up of MMN patients

Motor conduction block (MCB) has been used as the main diagnostic criterion in multifocal motor neuropathy (MMN). Nonetheless, no agreed definition of block currently exists; the proposed required percent decrement of proximal compound muscle action potential (CMAP) amplitude varies from > 20% to > 50%. The aim of this work was to evaluate, through a follow-up study of patients with MMN, the behaviour of MCB over time. The percent decrement and temporal dispersion of proximal CMAP have also been calculated in normal controls and in patients affected by amyotrophic lateral sclerosis (ALS). The results show that MCB in patients with MMN is a dynamic entity which greatly varies over time and that a > 50% CMAP amplitued reduction may well be preceded by a smaller decrement that is nonetheless indicative of focal myelin damage in the appropriate clinical context. This datum and the results obtained in the control group and in patients with ALS suggest that a reappraisal of the diagnostic criteria for MCB, in cases with clinical and electrophysiological data strongly indicative of MMN, should be considered. Since MMN is a treatable disorder, the use of the proposed less restrictive criteria for the identification of MCB could allow for a promp and more effective treatment. Received: 31 December 1996 Received in revised form: 1 August 1997 Accepted: 13 August 1997     

运动单位数目估计在肌萎缩侧索硬化患者随访中的作用

目的 比较多点刺激法和递增法运动单位数目估计在肌萎缩侧索硬化(ALS)患者随访中的作用及差异.方法 120例ALS患者在诊断时,随访3、6、9、12个月时分别进行多点刺激法或递增法运动单位数目估计.多点刺激法:刺激电极分别于腕、腕上6 cm、肘、肘上6 cm,4点刺激正中神经,以超强刺激诱发最大波幅M波;然后从0刺激开始逐渐增加刺激强度直到出现可辨认的单个运动单位电位,逐渐增大刺激强度,记录3个递增的M波.递增法:刺激电极于腕点刺激正中神经,以超强刺激诱发最大M波值,之后自阈强度刺激开始,逐渐增加刺激强度,收集10个递增的M波.比较两种方法在患者随访中所得运动单位数目估计数值的变化及差异.结果 在ALS患者诊断时,随访3、9、12个月时,两种方法所测运动单位数目无差异,均表现为进行性下降;在随访6个月时,多点刺激法所得数值高于递增法(88±6和47±5;t=1.72,P=0.04).结论 多点刺激法和递增法运动单位数目估计可用于ALS患者的随访研究,在疾病不同时期,两种方法所得数值可以不同.     

神经电生理检测对多灶性运动神经病诊断价值的初步研究

目的 探讨神经电生理检查在多灶性运动神经病(MMN)中的诊断价值。方法对16例MMN患者及16名健康对照进行运动神经传导速度和感觉神经传导速度检查，记录刺激引出的复合肌肉动作电位的波幅、波宽、面积、位相和时限，进行对比分析，判定是否有运动神经传导阻滞(CB)或暂时性离散(TD)，并有选择性地进行常规肌电图检查。结果16例患者均可见有一根以上运动神经或至少一根运动神经的一个以上部位出现CB或CD。其中13例双上肢正中神经，尺神经出现CB，3例以正中神经、尺神经的远端出现CB首发，随病情进展出现下肢腓深神经CB。仅有2例感觉神经传导速度稍有减慢，波幅略有降低。16例患者神经受累区域以下所支配肌肉肌电图检查见有神经源性损害。结论MMN是一种以远端神经受累为主的不对称性周围神经病，神经电生理检查对诊断和鉴别诊断．MMN起重要作用，CB是MMN的主要神经电生理表现。     

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.     

Significant CMAP decrement by repetitive nerve stimulation is more frequent in median than ulnar nerves of patients with amyotrophic lateral sclerosis

Introduction: Several studies have shown a significant amplitude decrement in compound muscle action potentials (CMAPs) on repetitive nerve stimulation (RNS) of muscles involved in amyotrophic lateral sclerosis (ALS).In ALS, muscle wasting preferentially affects the thenar muscles (APB) rather than the hypothenar muscles (ADM). Methods: We performed RNS studies in the APB and ADM muscles of 32 ALS patients to determine whether the effect of RNS differs between the median and ulnar nerves. Results: The decremental responses to RNS were greater in the APB than in the ADM. Reduced CMAP amplitude was negatively correlated with CMAP decrement in median but not in ulnar nerves. Conclusions: The greater CMAP decrement in median nerve is attributable to preferential involvement of the APB in the pathophysiology of ALS or some underlying difference in the biology of the two muscles/nerves. Further investigations will better our understanding of the pathophysiology of ALS. Muscle Nerve, 2012     

A new method for the estimation of motor nerve conduction block.

OBJECTIVE: A method for conduction block (CB) estimation, based on compound muscle action potentials (CMAP) elicited by stimulation at sites proximal and distal to the region in which a block is suspected, which is less sensitive to temporal dispersion than methods based on area and amplitude estimation, routinely used in clinical practice. METHODS: The method is based on deconvolution of CMAPs. It provides the delay distribution that convolved with a kernel (estimated by an optimisation method) gives a reconstruction of the CMAPs. The integral of the delay distribution was used to estimate CB. The method was tested on phenomenological signals (sum of delayed and amplitude scaled versions of the same signal), structure based simulated signals (from a plane layer generation model of surface EMG), and experimental signals (eight healthy subjects; CMAPs recorded over abductor digiti minimi; different temporal dispersions obtained comparing above-elbow stimulation of ulnar nerve with below-elbow stimulation or with wrist stimulation; conduction distances about 10 and 35 cm, respectively). RESULTS: Deconvolution method gives more precise estimates of the simulated CB with respect to area and amplitude methods (phenomenological signals: bias in CB estimation in the worst case about 10% for deconvolution, 30% for area, 60% for amplitude). Experimental data: by increasing temporal dispersion, in the average CB estimation increases 4% for area and 10% for amplitude, no increase for deconvolution. CONCLUSIONS: The new method for CB estimation is less sensitive to temporal dispersion than area and amplitude methods. SIGNIFICANCE: The proposed method provides a precise CB estimation. Being stable to temporal dispersion, it allows to diagnose CB with a lower confidence threshold than in the case of area and amplitude.     

Electrical stimulation over the human vertebral column: which neural elements are excited?

Supramaximal percutaneous electrical stimuli applied over the human cervical vertebral column produce maximal compound muscle action potentials (CMAPs) in abductor digiti minimi. It is important to know which neural elements are excited by these stimuli and experiments were performed to answer this question. With stimulating electrodes placed progressively lateral to the midline, submaximal CMAPs with the same latency are produced. With shocks over the cervical vertebrae in the midline, the threshold for excitation of arm muscles is much lower than for excitation of leg muscles. Comparison of conduction time from the cervical column to more distal sites on the ulnar nerve by direct measurement and by F wave latency determination shows that the latter exceeds the former by 1.6 msec. Collision experiments in which paired shocks were given at the wrist and Erb's point or the wrist and cervical column showed that recovery from blocking as interstimulus interval lengthened was similar for the two sites, and that it was possible to detect F waves from the proximal stimulus. The latency of CMAPs evoked from midline surface stimuli was identical to that from a needle stimulus near the C8 root. It is concluded that electrical stimuli applied over the cervical vertebrae in the midline excite the motor roots at their exit from the spinal canal. This finding has implications for clinical studies of pyramidal tract and proximal peripheral nerve conduction.     

Effects of prolonged repetitive stimulation of median,ulnar and peroneal nerves

It is important to know the effects of prolonged repetitive nerve stimulation (RNS) when it is used in neurophysiological studies. RNS with up to 100 supramaximal stimuli was given to the median, ulnar, and peroneal nerves of normal subjects and the ulnar nerves of subjects with early amyotrophic lateral sclerosis (ALS), recording evoked compound muscle action potentials (CMAPs). In all nerves, there was a decline in the CMAP area and a decrease in CMAP duration. For the peroneal nerve there was a decline in the CMAP amplitude, but a similar decline was not seen in the median or ulnar nerves. Cooling of the muscles resulted in decrement of both the amplitude and area with RNS. In ALS subjects, CMAP amplitude and area both declined after RNS of the ulnar nerve. In this study we describe the changes in CMAP with prolonged RNS among commonly tested normal nerves. Our findings have important implications with regard to RNS. Muscle Nerve, 2010     

Triple-stimulation technique in multifocal neuropathy with conduction block

In patients with multifocal neuropathy with conduction block (CB), CBs located between the root and Erb's point are not detected in nerve conduction studies. We therefore examined whether the triple-stimulation technique (TST) might provide a useful means of detecting CB proximal to Erb's point. Clinical assessments, extensive nerve conduction studies (NCS), conventional transcranial magnetic stimulation, and TST were performed on 10 patients with multifocal motor neuropathy with CB (MMNCB) and 6 patients with Lewis-Sumner syndrome. Conduction blocks located proximal to Erb's point were detected in 9 patients. Of the CBs, 58% were associated with muscle weakness. The use of TST to detect proximal CB improved the sensitivity of the American Association of Neuromuscular and Electrodiagnostic Medicine criteria for definite or probable MMNCB from 60% to 90%. Thus, the TST is a useful means for detection of proximal CB and gives NCS considerable additional diagnostic power.