EMG power spectrum, turns-amplitude analysis and motor unit potential duration in neuromuscular disorders

The diagnostic value of power spectrum analysis of the needle EMG pattern at a force of 30% of maximum was compared to that of turns-amplitude analysis and to that of manual measurements of motor unit potential (MUP) duration in the brachial biceps muscle of 20 patients with myopathy and 11 patients with neurogenic disorders. In myopathy the power spectrum analysis had the same diagnostic value as the turns-amplitude analysis and MUP duration measurements and the 3 methods supplemented each other. In patients with neurogenic disorders the diagnostic value of the power spectrum analysis as well as that of the turns-amplitude analysis were lesser than that of MUP duration measurement. In diseased muscles the amount of high frequencies increased with increasing ratio of turns to mean amplitude while there was no relation between the power spectrum and the MUP changes. The results suggest that the power spectrum analysis of EMG can be used as a diagnostic tool in patients with neuromuscular disorders.     

Sensitivity of motor unit potential analysis in facioscapulohumeral muscular dystrophy

Template-operated motor unit potential (MUP) analysis has made quantitative electromyography (EMG) feasible, even in busy laboratories, but validation of this approach is still necessary. In the present study, the utility of multi-MUP analysis was assessed in patients with a molecular genetic diagnosis of facioscapulohumeral muscular dystrophy (FSHD). Manual assessment of muscle strength and concentric-needle EMG of the biceps brachii and vastus lateralis muscles were performed. The sensitivity for diagnosing myopathy (mean values and outliers) was tested for eight MUP parameters and four of their combinations. The group comprised 31 patients. Elbow flexion and knee extension strength was normal in 45% and 52% of patients, respectively. The most sensitive MUP parameter was thickness, followed by duration. A combination of three MUP parameters (thickness, amplitude, and duration/area) was needed for maximal sensitivity. The study demonstrated a high sensitivity of multi-MUP analysis in FSHD. Myopathic abnormalities were demonstrated in all weak biceps brachii muscles, and in 77% of biceps brachii muscles with normal strength.     

The motor unit firing rate and the power spectrum of EMG in humans

The EMG power spectrum is influenced by many factors such as the conduction velocity of the muscle fiber, the action potential of the motor unit, the number of motor units firing near the electrode, and the recording conditions. Model studies of the relation between motor unit firing rate and power spectrum of EMG have produced conflicting results. To examine this relation in vivo the brachial biceps muscle was examined in 14 controls at a force of 10% of maximum. The motor unit firing intervals were obtained from 164 motor units, sampled with a single fiber electrode. The EMG was sampled at 10 sites in each muscle with a concentric electrode and the power spectrum was obtained using fast Fourier transformation. The mean power frequency of the interference pattern as well as the relative power at 1400 Hz both decreased with increasing motor unit firing intervals between subjects. The study thus indicates that the amount of high frequencies in the power spectrum is greater in a subject with a high firing rate of the motor units than in a subject with a low firing rate.     

Quantitative motor unit analysis: the effect of sample size.

This study of quantitative electromyography examines the influence of sample size on motor unit action potential (MUAP) tolerance limits, intertrial variability, and diagnostic sensitivity. We recorded 20 randomly selected MUAPs from the biceps muscle twice in 21 normal subjects, and once in 10 patients with myopathy. The 95% tolerance limits for mean total duration in normal subjects progressively narrowed from 6.6 to 14.2 ms for 5 MUAPs to 7.4 to 13.0 ms for 20 MUAPs. The 95% tolerance limits for intertrial variability were +/-22% for mean total duration of 20 MUAPs. Larger sample size had a greater effect on reducing intertrial variability than on narrowing 95% tolerance limits for amplitude and area. Quantitative EMG results for duration supported the presence of myopathy in 2 of 10 patients with analysis of 5 MUAPs, and 9 patients with analysis of 20 MUAPs. Although analysis of 5 potentials may be adequate for diagnosis occasionally, quantitative analysis of 20 MUAPs narrows tolerance limits, reduces intertrial variability, and improves diagnostic sensitivity.     

Motor unit firing intervals and other parameters of electrical activity in normal and pathological muscle

The analysis of the firing intervals of motor units has been suggested as a diagnostic tool in patients with neuromuscular disorders. Part of the increase in number of turns seen in patients with myopathy could be secondary to the decrease in motor unit firing intervals at threshold force of the motor units, as noted in previous studies. In the brachial biceps muscle we have studied the firing intervals of 164 motor units in 14 controls, 140 motor units in 13 patients with myopathy and 86 motor units in 8 patients with neurogenic disorders, and related the findings to those of the turns analysis and the analysis of properties of individual motor unit potentials. To ensure comparable conditions we have examined motor unit firing intervals and turns at a force of 10% of maximum. The average of motor unit firing intervals and of interval variability was the same in controls and in patients, and the diagnostic yield of the motor unit firing intervals analysis was none. Although the number of turns increased with decreasing motor unit firing intervals, this relation was physiological rather than pathophysiological. In patients with neurogenic disorders, interval variability indicated unstable firing in severely affected muscles.     

Cytoplasmic body myopathy masquerading as motor neuron disease

Cytoplasmic body myopathy (CBM) is characterized by proteinaceous inclusion bodies in muscle tissue. A 43-year-old woman presented with rapidly progressive weakness and dysphagia. Electromyography (EMG) elsewhere demonstrated lower-limb chronic partial denervation. Muscle biopsy showed fiber size variation without diagnostic features. A diagnosis of possible motor neuron disease was made and the patient was commenced on riluzole. Subsequently, the patient's condition stabilized, prompting reassessment. Repeat EMG demonstrated no features of denervation and was more suggestive of a myopathic process. Review of the original muscle biopsy showed cytoplasmic bodies. The case highlights a further diagnostic possibility in the assessment of patients with "possible" motor neuron disease. The clinical features of CBM are briefly reviewed.     

Diagnostic yield of muscle fibre conduction velocity in myopathies

We prospectively assessed diagnostic yield of muscle fiber conduction velocity (MFCV) studies in patients with signs and symptoms suggestive of a myopathy. Results were analysed with respect to the final diagnosis, and compared to the reference standard, which was qualitative electromyography (EMG), turns-amplitude analysis (TAA), and muscle biopsy. We included 125 patients, in whom a myopathy was diagnosed in 71, and a neuromuscular disorder was excluded in 54. Sensitivity of MFCV for the presence of a myopathy was 84%, and specificity 83%. Diagnostic yield of MFCV was superior to EMG, TAA, and muscle biopsy in patients with metabolic myopathies, non-dystrophic myopathies, and channelopathies. We concluded that measurement of MFCV is a quantitative EMG technique with a high diagnostic yield. In certain myopathies, MFCV may be more informative than conventional EMG examination.     

Electromyography (EMG) accuracy compared to muscle biopsy in childhood

Reports show wide variability of electromyography (EMG) in detecting pediatric neuromuscular disorders. The study's aim was to determine EMG/nerve conduction study accuracy compared to muscle biopsy and final clinical diagnosis, and sensitivity for myopathic motor unit potential detection in childhood. Of 550 EMG/nerve conduction studies performed by the same examiner from a pediatric neuromuscular service, 27 children (ages 6 days to 16 years [10 boys; M:F, 1:1.7]) with muscle biopsies and final clinical diagnoses were compared retrospectively. Final clinical diagnoses were congenital myopathies (5 of 27,18%), nonspecific myopathies (biopsy myopathic, final diagnosis uncertain; 6 of 27, 22%), congenital myasthenic syndrome (3 of 27, 11%), juvenile myasthenia gravis (1 of 27, 4%), arthrogryposis multiplex congenita (2 of 27, 7%), hereditary motor and sensory neuropathy (1 of 27, 4%), bilateral peroneal neuropathies (1 of 27, 4%), and normal (8 of 27, 30%). There were no muscular dystrophy or spinal muscular atrophy patients. EMG/nerve conduction studies had a 74% agreement with final clinical diagnoses and 100% agreement in neurogenic, neuromuscular junction, and normal categories. Muscle biopsies concurred with final diagnoses in 87%, and 100% in myopathic and normal categories. In congenital myasthenic syndrome, muscle biopsies showed mild variation in fiber size in 2 of 3 children and were normal in 1 of 3. EMG sensitivity for detecting myopathic motor unit potentials in myopathies was 4 of 11 (36%), greater over 2 years of age (3 of 4, 75%), compared to infants less than 2 years (1 of 7, 14%), not statistically significant (P = .0879). EMGs false-negative for myopathy in infants < 2 years of age were frequently neurogenic (3 of 6, 50%). In congenital myopathies EMG detected myopathic motor unit potentials in 40%, with false-negative results neurogenic (20%) or normal (40%). Because our study has no additional tests for active myopathies, for example Duchenne muscular dystrophy genetic testing, our sensitivity for myopathies is lower than if we used a more global view. In conclusion, EMG detection rate of myopathic motor unit potentials at a young age was low, improving in children over 2 years of age. In neurogenic and neuromuscular junction disorders, the EMG has a very high detection rate. In children with mild to moderate neurogenic EMG findings and normal nerve conduction, a myopathy should always be considered.     

Electromyographs of the flexor digitorum profundus muscle are useful for the diagnosis of inclusion body myositis

Introduction: The frequent observation of high‐amplitude and long‐duration motor unit potentials (MUPs) in inclusion body myositis (IBM) is problematic, because it may lead to a misdiagnosis of amyotrophic lateral sclerosis (ALS). Objective: To document the diagnostic utility of EMG from the flexor digitorum profundus (FDP) muscle for IBM. Methods: Quantitative analyses of MUP parameters were performed in the FDP and biceps brachii (BB) muscles from 7 biopsy‐confirmed IBM patients. Results: In the FDP muscle, all MUP parameters were significantly decreased in IBM patients, which indicated the predominance of low‐amplitude and short‐duration MUPs in this muscle. In the BB muscle, most parameters were increased, suggesting the frequent contamination of high‐amplitude and long‐duration MUPs. Conclusions: Low‐amplitude MUPs in the FDP muscle indicate the presence of an advanced myopathy in this muscle that was extremely weak for all subjects. Examining the FDP muscle would reduce the chance of misdiagnosing IBM as ALS. Muscle Nerve 46: 181–186, 2012     

Diagnostic yield of analysis of the pattern of electrical activity and of individual motor unit potentials in myopathy.

The analysis of the pattern of electrical activity and of individual motor unit potentials in the same muscle both identified about 90% of 41 patients as having a myopathy. The pattern of electrical activity was analysed during a force which was a fixed fraction of maximum; individual motor unit potentials were analysed during weak effort. The two methods supplement each other as some of the patients were identified only by one or by the other of the two procedures. The parameter of the pattern of electrical activity which was most often abnormal was the ratio: numbers of turns to mean amplitude between turns.     

Origin of ICU acquired paresis determined by direct muscle stimulation

BACKGROUND: Acquired diffuse paresis in an intensive care unit (ICU) can result from critical illness myopathy or polyneuropathy. Clinical examination and conventional neurophysiological techniques may not distinguish between these entities. OBJECTIVE: To assess the value of direct muscle stimulation (DMS) to differentiate myopathic from neuropathic process in critically ill patients with diffuse severe muscle weakness. METHODS: 30 consecutive patients with ICU acquired diffuse motor weakness were studied. Responses of the right deltoid and tibialis anterior muscles to DMS and to motor nerve stimulation (MNS) were studied and compared with results of conventional nerve conduction studies and concentric needle electromyography (EMG). An original algorithm was used for differential diagnosis, taking into account first the amplitude of the responses to DMS, then the MNS to DMS amplitude ratio, and finally the amplitude of the sensory nerve action potentials recorded at the lower limbs. RESULTS: Evidence of neuropathy and myopathy was found in 57% and 83% of the patients, respectively. Pure or predominant myopathy was found in 19 patients. Other results were consistent with neuromyopathy (n = 5) and pure or predominant neuropathy (n = 2). Four patients had normal results with stimulation techniques, but spontaneous EMG activity and raised plasma creatine kinase suggesting necrotic myopathy. CONCLUSIONS: A neurophysiological approach combining DMS and conventional techniques revealed myopathic processes in a majority of ICU patients. Reduced muscle fibre excitability may be a leading cause for this. The diagnosis of myopathy in ICU acquired paralysis can be established by a combination of DMS, needle EMG, and plasma creatine kinase.     

Reliability of EMG determinism to detect changes in motor unit synchrony and coherence during submaximal contraction

The determinism (DET) is a parameter used in nonlinear analysis to quantify the occurrence of recurrent patterns in a signal. Applied to the electromyographic activity (EMG), DET has been proposed as an index of motor unit synchrony in human. We have recently shown that the amount of motor unit synchronous firings above chance level was enhanced with stronger submaximal muscle contraction. Using these data, we aimed at determining if (1) EMG DET and motor unit synchrony varied in the same way and (2) EMG DET was more specifically related to the degree of oscillatory coupling between motor unit discharges. Cross-correlation and coherence analyses were applied to the discharges of 30 motor unit pairs tested at various force levels to assess the amount of synchronous impulses and the strength of oscillatory coupling in the time and frequency domains, respectively. Recurrent quantification analysis was applied to EMG activity to extract its DET. Overall, changes in EMG DET were poorly explained by changes in motor unit synchronous impulse probability (6%) and frequency (5%), and by changes in motor unit coherence in the 6-12Hz (5%) and 25-40Hz (8%) bands. Moreover, the comparison of the data obtained at the weakest and the strongest contraction levels tested with each motor unit pair showed that EMG DET remained unaltered with stronger contraction despite the occurrence of consistent changes in motor unit synchrony in both time and frequency domains. This speaks strongly against the reliability of DET in evaluating changes in motor unit synchronization during submaximal muscle contraction.     

The motor unit and quantitative electromyography

Electromyography (EMG) assesses the anatomic motor unit (A-MU), but knowledge of its anatomy, physiology, and changes with pathology is limited. The electrophysiological motor unit (E-MU) and its motor unit potential (E-MUP) represents a fraction of the A-MU. Routine EMG assesses a limited number of E-MUP waveform characteristics (metrics) and their magnitudes qualitatively scaled in a nonlinear manner. Another approach is quantitative EMG (QEMG), whereby 20⁺ E-MUPs are extracted and both basic and derived metrics obtained and values expressed quantitatively. In diseased muscle, many E-MUP metrics may be normal, which complicates diagnostic interpretation. In QEMG, E-MUP metrics can be clustered and statistical analyses performed to assign probabilities that E-MUPs (and the muscle) are normal, neuropathic, or myopathic. In this article we review what is known about the A-MU, the restricted E-MU, E-MUP metrics, and what QEMG offers currently and in the future.     

Electrophysiological findings including single fibre EMG in a family with mitochondrial myopathy

Nerve conduction studies, conventional and quantitative concentric needle EMG and single fibre EMG were performed on 5 clinically affected and 7 clinically asymptomatic membres of a family with a mitochondrial myopathy manifesting as a facioscapulohumeral syndrome. Abnormalities of nerve conduction present in 3 clinically affected cases were attributed to co-existent diabetes mellitus. Quantitative CNEMG showed a reduction of the mean motor unit potential duration and increased incidence of polyphasic potentials in all 5 clinically manifest cases consistent with a primary myopathic disorder. Similar but less marked changes were found in 6 of the clinically asymptomatic individuals revealing the presence of a subclinical myopathy. Abnormalities on SFEMG consisting of increases in fibre density and/or jitter were present in all the clinically affected and in 5 clinically normal cases. These changes indicate local reorganization of the spatial arrangement of muscle fibres of the motor unit and a disturbance of neuromuscular transmission. The CNEMG and SFEMG findings are discussed in relation to the histopathological changes in 4 cases.     

98例神经肌肉病的临床、肌电图与病理研究

目的 探讨肌电图（EMG），肌活检对神经肌肉病的诊断价值。比较EMG，肌活检及初始临床诊断3者之间的关系。方法 将98例神经肌肉病分成肌病。重症肌无力和运动神经元病3组进行研究。结果 肌病组（80例），68.8%(55/80)肌活检，75%（60/80）EMG呈肌源性损害；重症肌无力组（10例）；针极EMG（不包括重视频率电刺激）及肌活检均未显示特异性改变；运动神经元病组（8例），75?/8）肌活检，100%（8/8）EMG呈神经源性损害。结论 肌活检对肌病明确诊断可提供直接信息。对运动神经元病只能做出神经源性损害结果。缺乏特异性。EMG对神经肌肉病只能做出分类诊断；单纯凭借初始临床资料易导致该类疾病误诊。     

Techniques and applications of EMG: measuring motor units from structure to function

Needle electromyography (EMG) is an established method of evaluating motor unit and muscle fibre function and pathology in clinical practice, while the development of advanced techniques including single-fibre EMG and combined recordings with other modalities have become increasingly useful in research. The development of quantitative EMG in particular had led to greater reproducibility and inter-rater reliability. This review provides an overview of standard needle EMG as well as discussing advanced recording and analysis techniques and their increasing role in clinical research.     

Acoustic myography: A noninvasive monitor of motor unit fatigue

Acoustic myography is the recording of sounds produced by contracting muscle. These sounds become louder with increasing force of contraction. We have compared muscle sounds with surface EMG to monitor the dissociation of electrical from mechanical events (presumably, the loss of excitation–contraction coupling) which occur with motor unit fatigue. Acoustic signals were amplified using a standard phonocardiograph, recorded on FM magnetic tape, and digitally analyzed. Muscles were examined at rest, with intermittent contractions, and with sustained contractions. We found that with fatigue, the acoustic amplitude decayed, but the surface EMG amplitude did not. With decreased effort, however, the acoustic and the surface EMG amplitudes declined simultaneously. By simultaneously recording acoustic signals and needle EMG, individual motor units were resolved acoustically in two muscles with decreased numbers of motor units and increased motor unit size. Fasciculations also produced acoustic signals, although no acoustic signal has yet been found that correlates with fibrillations. Analysis of acoustic signals from muscle provides a noninvasive method for monitoring motor unit fatigue in vivo. It may also be useful in distinguishing muscle fatigue from decreased volition.     

Clinicopathological phenotype of ALS with a novel G72C SOD1 gene mutation mimicking a myopathy

A 71-year-old woman with a family history of amyotrophic lateral sclerosis (ALS) was investigated for symmetrical, proximal limb and abdominal muscle weakness. Initial examination showed mild proximal muscle weakness in the arms and legs, slightly elevated serum creatine kinase (CK) level, and normal electromyographic (EMG) findings. A myopathy was the presumed diagnosis. Over the next year, weakness became severe and tendon reflexes became unelicitable; no upper motor signs were present. EMG then showed acute and chronic denervation and a muscle biopsy showed target fibers and grouped atrophy. DNA analysis revealed a G72C CuZn-superoxide dismutase (SOD1) mutation. Fasciculations were absent throughout the disease. The patient died 53 months after symptom onset and autopsy revealed loss of lower motor neurons (LMN) and SOD1-positive inclusions. This case expands the phenotypic spectrum of ALS associated with SOD1 mutations to include presenting features that mimic a myopathy.     

Electromyographic evidence of subclinical myopathy in hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is due to a number of mutations of contractile protein genes such as beta-cardiac myosin, myosin binding protein-C, and troponin-T. Unlike troponin-T, beta-myosin is a constituent of slow skeletal muscle and its mutations generally have a better prognosis. In order to investigate the usefulness of electromyography in detecting skeletal muscle involvement in HCM, 46 patients were examined using both conventional electromyography (EMG) and quantitative electromyography (QEMG) methods. The QEMG involved motor unit potential (MUP) analysis, turns/amplitude (TAA) analysis, and power spectrum analysis of the interference pattern. Using conventional EMG, myopathic findings were demonstrated in 13 patients (28%). Receiver operating characteristic (ROC) analysis of the results of a discriminant function extracted using QEMG values, identified correctly 10 out of 11 normal controls and all 9 myopathic control patients, and displayed a 15% presence of myopathy (7 patients) among the cardiomyopathy group. The duration of MUPs was the most sensitive among the quantitative parameters in differentiating normal from myopathic subjects. Since skeletal muscle involvement may be due to distinct gene mutations, normal and myopathic EMG findings may reflect HCM subpopulations with a different genetic substrate.