Quantitative electrophysiologic studies in sporadic inclusion body myositis

Sporadic inclusion body myositis (S-IBM) is a progressive, acquired disease of unknown etiology. Prior studies have suggested neurogenic involvement based on electrophysiologic data, although the biopsy is compatible with a myopathic process. Quantitative electrophysiologic studies were performed in the biceps brachii of 17 subjects with biopsy-proven S-IBM. Quantitative motor unit action potential (MUAP) analysis was compatible with myopathy in 16 subjects, with the remaining subject being within normal limits. Quantitative interference pattern was myopathic in all 13 subjects studied. Macro-EMG MUAP amplitude was reduced in 3 of 17 studies; the remainder were within normal range, and none was increased as would be expected in neurogenic disease. Fiber density was normal to borderline increased in all subjects. Possible reasons for encountering neurogenic-appearing MUAPs may include choice of muscle studies, because some patients have co-existing polyneuropathy and large-amplitude MUAPs from hypertrophied muscle fibers. The data from this study indicate that S-IBM is a myopathic process.     

Simulation and analysis of the electromyographic interference pattern in normal muscle. Part II: Activity, upper centile amplitude, and number of small segments

We have defined three new features of the electromyographic (EMG) interference pattern (IP): activity, upper centile amplitude (UCA), and number of small segments (NSS). These parameters were measured in simulated IPs constructed by adding together motor unit action potentials (MUAPs) recorded with a concentric needle EMG electrode. The activity increases linearly with the number of MUAP discharges to approximately 80% of its theoretical maximum value. The UCA correlates strongly with the peak-to-peak amplitude of the largest MUAP in the IP and the mean segment amplitude and does not depend on the discharge rate of the largest MUAPs. We infer that the UCA defines the upper limit of the peak-to-peak amplitude of the MUAPs contained in the IP. The NSS increases with the number of MUAP discharges, but reaches a constant value at higher MUAP discharge rates, probably because small amplitude MUAPs are masked by the large amplitude MUAPs. The potential value of these parameters in automated IP analysis is discussed.     

Simulation and analysis of the electromyographic interference pattern in normal muscle. Part I: Turns and amplitude measurements

The electromyographic (EMG) interference pattern (IP) was simulated by adding together motor unit action potentials (MUAPs) of different sizes that had been recorded by a concentric needle EMG electrode. The number of turns (NT) of the simulated IP increased with the number of MUAP discharges. The mean amplitude (MA) difference between successive turns in the IP increased when large amplitude MUAPs were added. Our analysis demonstrates that the MA of the IP is determined mainly by the amplitude of large MUAPs in the signal and that large amplitude spikes are more likely to be generated by single large amplitude MUAPs than by summation of several small amplitude MUAPs.     

Quantification of motor unit action potential energy

ObjectiveMotor unit action potentials (MUAPs) recorded by needle electrode reflect the functional state of the motor unit and its force-generating capacity, and are usually described morphologically (e.g. amplitude, duration). However, since the purpose of motor unit activation is force generation, MUAP energy seems a more physically meaningful measurement.MethodsMUAPs were obtained by multi-MUAP decomposition of real interference patterns taken from human patients with neurological diseases. The energy content of each MUAP was measured from a time–frequency representation (TFR), specifically the Choi–Williams distribution, and compared with the standard MUAP morphological measure, the Size Index. The sample included normal, neurogenic, and myopathic MUAPs, from 11 patients.ResultsThere is an exponential distribution of energy within a sample of MUAPs and a strong exponential relationship between the Size Index and MUAP energy was observed.ConclusionsThe energy content of a MUAP can be quantified and corresponds very well with the current quantitative standard. Energy is a possible addition to MUAP quantification.SignificanceMUAPs could be classified as having normal, large (neurogenic), or low (myopathic) energy. MUAP energy has direct physical and physiological meaning that reflects the force-generating capacity of the motor unit. Time–frequency analysis could also be used to study the specific frequency content of MUAPs and the energy of MUAPs within an interference pattern, without the need for decomposition.     

Multi-motor unit action potential analysis (MMA)

We have developed an algorithm, called multi–motor unit action potential analysis (MMA), to aid quantification in routine needle EMG examination. In only 5–8 min, it was possible to extract, analyze, and validate 20 motor unit action potentials (MUAPs). In the biceps muscle of normal subjects, the MUAP measurements are compared with measurements using manual, triggered averaging, automatic decomposition, and other MMA algorithms described in the literature. The mean values of MUAP features by MMA fell between the extreme limits obtained from the amplitude-triggered method and the manual method described in the literature. In patients, the results are consistent with routine EMG and similar to those results described in the literature. Fast acquisition and analysis of MUAPs as well as interference pattern (IP) analysis make this technique useful to document EMG abnormalities in routine needle examination.© 1995 John Wiley & Sons, Inc.     

Automatic decomposition electromyography (ADEMG): validation and normative data in brachial biceps

We describe a new, automatic method (ADEMG) for decomposing EMG interference patterns into their constituent motor unit action potentials (MUAPs), and quantitating the configurational and firing properties of the MUAPs. ADEMG is fast (90 sec analysis time for a 10 sec EMG epoch) and efficient (33-98% of MUAP occurrences correctly identified) because of 4 signal-processing innovations designated digital prefiltering, high-resolution wave form alignment, firing-time analysis and interference-cancellation averaging. Validation experiments are described involving recruitment/derecruitment of low-threshold MUAPs, and single-fiber-triggered averaging. Normative data are presented for 2000 MUAPs from brachial biceps (mean 9.7 MUAPs per site) at 3 levels of isometric contraction (7%, 15% and 30% MVC) and contrasted with normal findings obtained using traditional, low-threshold MUAP analysis. The main advantages of ADEMG are speed of data acquisition and processing in the clinical setting; ability to analyze both low- and high-threshold MUAPs during moderately strong muscular contractions; and MUAP firing-rate information.     

A comparison of turns analysis and motor unit analysis in electromyography.

We compared the results of turns analysis and motor unit analysis on 4056 electromyographic interference patterns (IPs) from normal subjects and patients with neuromuscular disorders. The motor unit analysis involved decomposing the IPs into their component motor unit action potentials (MUAPs) using automatic decomposition electromyography (ADEMG). We checked the accuracy of the decompositions by attempting to reconstruct some of the IPs from their identified MUAPs using computer simulations. The simulations revealed that ADEMG typically identified more than 60% (but not all) of the MUAPs in a given IP. Both turns and MUAP properties showed regular and related changes with force, age, muscle, and recording electrode type. The number of turns in each IP was highly correlated with the number of active MUAPs (r = 0.65), the mean MUAP firing rate (r = 0.72), the mean number of turns per MUAP (r = 0.34), and the product of these 3 properties (r = 0.83). The mean amplitude change per turn was highly correlated with the mean MUAP amplitude (r = 0.82), but also depended on the number of turns per MUAP. Due to the lack of a one-to-one relationship between the turns analysis properties and the MUAP properties, the turns analysis properties by themselves did not provide sufficient information to infer unambiguous physiological information about motor unit morphology or firing behavior.     

Anal sphincter electromyography: editing of sampled motor unit action potentials

During multi-motor unit action potential (MUAP) analysis of the tonically contracted external anal sphincter (EAS), a mild interference pattern often obscures the baseline, affecting the algorithm's ability to determine accurate boundaries of detected MUAPs. To assess the equivalence of methods of editing and selecting candidate MUAPs from the EAS, 17 nulliparous women underwent concentric needle electromyography (EMG) of the EAS using multi-MUAP software. The selected MUAPs either were accepted without question ("automated"), or a subset was deleted due to a noisy baseline ("manual-deletion") or manually marked ("manual-mark"). A second examiner repeated the analysis. Each examiner found that the two editing methods were equivalent and yielded results that differed from those obtained by automated analysis of unedited data. However, there was a moderate difference in MUAP amplitude when the manual-deletion method was compared between the two examiners. Editing of selected EAS MUAPs during multi-MUAP analysis is required, and development of common protocols is essential to enable meaningful comparisons between similar studies.     

Recording characteristics of monopolar EMG electrodes

Motor unit action potentials (MUAPs) and the electromyographic (EMG) interference pattern (IP) were recorded from the biceps muscle of 5 normal subjects using both a concentric needle (CN) and a disposable monopolar needle (MN) electrode. The MUAPs recorded by the MN electrode had higher amplitude and area and were more frequently complex than those recorded with the CN electrode. The MUAP duration and area: amplitude ratio were similar for both electrodes. Although the MN electrode had a larger recording surface, its dimensions (maximum diameter and length of the cone shaped tip) were similar to those of the CN electrode (minor and major axes of the elliptical recording tip). Based on these observations, we infer that the MN electrode may be more selective than the CN electrode, ie, the AP amplitude recorded by the MN electrode decreases faster than the AP amplitude recorded by a CN electrode when the distance of the muscle fiber from the recording electrode increases. Photomicrographs of the MN electrode after use demonstrated no evidence that the insulating material had peeled off. There was also no evidence that MUAP measurement values changed during the recordings as would be expected if the recording surface changed due to peeling of the insulating material.     

Age effects on properties of motor unit action potentials: ADEMG analysis

We have measured the configurational and firing properties of 13,206 motor unit action potentials (MUAPs) from the brachial biceps, brachial triceps, and anterior tibial muscles in 10 young (20-40 years), 10 middle-aged (40-60 years), and 10 elderly (60-80 years) normal individuals, using an automatic method for decomposition of the electromyographic (EMG) interference pattern (ADEMG). Recording were made during stable isometric contractions at threshold, 10%, and 30% of maximum voluntary contraction using standard concentric needle electrodes. At supra-threshold forces, an average of 5.9 simultaneously active MUAPs were identified at each recording site. Mean amplitudes, durations, and numbers of turns all increased linearly with age in both low-threshold and high-threshold MUAPs (p less than 0.01), suggesting an ongoing process of progressive denervation and compensatory reinnervation. Mean MUAP firing rates decreased with age (p = 0.01) when force was measured proportionately, but not when measured absolutely. In a subgroup of 12 age-matched gender pairs, men had larger mean MUAP amplitudes, rise rates, and numbers of turns (p less than 0.05), probably reflecting larger muscle fiber diameters. These findings amplify previous observations from traditional analysis of lowest-threshold single MUAPs; establish a base of normative adult data for ADEMG; and further validate the clinical applicability of rapid, automatic EMG decomposition.     

Evaluation of an automatic method of measuring features of motor unit action potentials

This study was performed to evaluate an automatic method of motor unit action potential (MUAP) analysis developed in our laboratory. MUAPs were recorded from the biceps brachii muscle of 68 normal subjects and 122 patients with nerve or muscle disease. The values of mean MUAP durations from normal subjects obtained by automatic analysis were similar to those reported in the literature. However, the normal range of MUAP amplitude and the incidence of polyphasic MUAPs were much higher. Normal ranges of mean MUAP area, area/amplitude ratio, and the number of turns were also defined. Automatic analysis demonstrated an abnormality of at least one MUAP feature in 70% of patients. There was concordance between automated analysis and visual assessment of MUAPs in 76% of patients with neuropathy but in only 50% of patients with myopathy. The relationships between different MUAP features seen in neuropathy and myopathy are explained in physiologic terms.     

Needle electromyography of the rectus abdominis in patients with amyotrophic lateral sclerosis

We examined the role of needle electromyography (EMG) of the rectus abdominis (RA) in assessing thoracic involvement in amyotrophic lateral sclerosis (ALS). Needle EMG of the RA was performed in 67 patients with sporadic ALS and 110 healthy controls. The presence of abnormal spontaneous activity, configuration of motor unit action potentials (MUAPs), and recruitment pattern of motor unit potentials were examined. In ALS patients, MUAPs in the RA were of prolonged duration, large amplitude, and showed increased prevalence of polyphasic waveforms compared to controls. Significant differences in MUAP parameters, presence of abnormal spontaneous potentials, and interference patterns were noted between ALS patients and controls. Additionally, we found that active denervation was more frequent in the RA of ALS patients with dyspnea than those without dyspnea. Thus, conventional needle EMG of the RA is a valuable electrophysiological method to assess clinical and subclinical involvement of thoracic lower motor neurons in patients with suspected ALS.     

Motor unit firing rates and firing rate variability in the detection of neuromuscular disorders

We have used automatic decomposition electromyography (ADEMG) to study 41 muscles in 29 patients with well-defined peripheral and central motor disorders. In motor neuron diseases motor unit action potentials (MUAPs) showed increased amplitudes, firing rates and firing variability. Relatively large MUAPs sometimes were not identified by the computer program if they lacked sufficient high-frequency signal content, or were too variable in shape. In myopathies the MUAPs showed reduced amplitudes, durations and turns, and sometimes dramatic increases in firing rates. Also, the mean number of MUAPs per recording site was often increased, indicating excessive recruitment. In polymyositis (the best studied myopathy) the nature and magnitude of the MUAP shape and firing abnormalities were usually similar at different levels of contractile force, suggesting that motor units are affected without regard to recruitment order. In upper motor neuron paresis (multiple sclerosis), the shape properties of the MUAPs were normal, but mean firing rates were reduced, and firing variability increased. These findings confirm many of the traditional criteria for distinguishing neurogenic from myopathic disease electrophysiologically at the level of the individual MUAP. In addition, they demonstrate the potential diagnostic sensitivity of MUAP firing rate measurements for detecting neuromuscular dysfunction, and for differentiating between some cases of central and peripheral paresis, but not for distinguishing peripheral neurogenic from myopathic weakness, since firing rates tend to increase in both. Increased firing rate variability may be a marker of central or peripheral neurogenic weakness.     

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.     

EMG of reinnervated motor units: a simulation study

Using computer simulation techniques, reinnervation of motor units (MUs) was studied by increasing the number of muscle fibers in the MU without changing the MU territory. The fiber density (FD) measured by single fiber EMG electrodes, the amplitude, area and number of turns of concentric needle (CN) EMG motor unit action potentials (MUAPs) and the amplitude of macro EMG MUAPs were most affected by partial reinnervation changes. The values of these features increased during simulated advanced reinnervation, as did the number of CNEMG MUAPs that had increased numbers of phases or turns and the mean CNEMG MUAP duration. The increase in macro EMG MUAP amplitude, FD and CNEMG MUAP area were proportional to the increase in the number of muscle fibers in the MU. When loss of muscle fibers due to so-called MU fractionation was simulated, values of all EMG features fell, but were still increased compared to normal. Two patterns of change in SFEMG and macro EMG values were identified that may distinguish between recordings made from reinnervated low force threshold MUs and those from higher force threshold MUs.     

Comparison of standard and pediatric size concentric needle EMG electrodes.

OBJECTIVE: To compare motor unit action potential (MUAP) metrics recorded by standard and pediatric size concentric EMG electrodes. METHODS: Commercial electrodes were used to record MUAPs from biceps brachii, first dorsal interosseous and tibialis anterior muscles in normal subjects and those with amyotrophic lateral sclerosis (ALS). RESULTS: In normal subjects, peak amplitude and area were significantly higher when recorded by the pediatric size electrode in tibialis anterior muscles and peak amplitude recorded in first dorsal interosseous muscles. In ALS subjects, peak amplitude was higher recorded by the pediatric size electrode in tibialis muscle but lower when recorded in first dorsal interosseous muscles. CONCLUSIONS: Differences of MUAP metrics when recording with standard and pediatric size electrodes do not seem to have a clinical relevance. SIGNIFICANCE: Pediatric and standard concentric electrodes record similar MUAP metrics.     

Simulation of concentric needle EMG motor unit action potentials

Computer simulations of motor unit action potentials (MUAPs) as measured by a concentric needle (CN) electromyography (EMG) electrode in normal motor units (MUs) indicated that the MUAP amplitude is determined mainly by the proximity of the electrode to the closest muscle fiber. The area and duration of the simulated MUAPs were affected by all muscle fibers in front of the active recording surface but mainly by those that were less than 2 and 2.5 mm, respectively, from the active recording surface. The MUAP area was also affected by the proximity of the electrode to the closest muscle fiber. The number of phases of the simulated MUAPs increased when the dispersion of the arrival times of individual muscle fiber APs at the electrode was increased. Increased temporal dispersion of APs decreased the MUAP amplitude and area slightly but did not affect the MUAP duration. It is inferred that different features of the CN MUAP are determined by the distribution of muscle fibers within different portions of the MU territory and thus provide complementary information about the MU architecture.     

Antigravity posture for analysis of motor unit recruitment: the "45 degree test"

The maximum number of different motor unit action potentials (MUAPs), their firing rates, and total MUAP spikes/second recorded by monopolar needle electrode were determined for the biceps brachii muscle during 45-degree elbow flexion. There were 4.2 +/- 1.6 different MUAPs exceeding 100 microV. Mean firing rate was 10.0 +/- 1.7 Hz, and total MUAP spikes/second were 40.3 +/- 18. Recordings from 16 patients with neurogenic atrophy (NA) and just detectable weakness revealed corresponding values of 3.1 +/- 1.7 different MUAPs, a mean rate of 10.2 +/- 1.5 Hz and 30.6 +/- 19 total MUAP spikes/second, not different from normal. In these patients, increased force of muscle contraction was required to activate high threshold motor units firing at high rates. In each of 4 patients just able to hold the arm against gravity, 1 or 2 "overdriven" motor units firing at a mean rate greater than 20 Hz were recorded. In 8 patients with myopathy and just detectable weakness, greater than 100 total MUAP spikes/second were recorded. Antigravity posture as a reference level of innervation has the advantage that motor unit firing rate is set about that of physiologic tremor (10-13 Hz). Its application was helpful in quantifying recruitment.     

Multielectrode surface EMG for noninvasive estimation of motor unit size.

To noninvasively estimate the motor unit size, we present a novel surface electromyographic (EMG) measurement system consisting of a surface multielectrode with four-pin electrodes and a pair of surface-disk electrodes. Surface motor unit action potentials (MUAPs) were recorded with the multielectrode, in the so-called multielectrode surface EMG (MSEMG), which was spatially filtered to localize the sensing area and reduce the noise. In addition, a modified decomposition algorithm, considering the geometrical configuration of the multielectrode, was designed to identify the individual MUAPs in the measured MSEMG. The identified MUAP was subsequently used as the triggering source for the EMG signals recorded by the surface-disk electrodes. From a pool of 34 subjects with neuromuscular diseases and 14 normal subjects, the median amplitudes of surface-disk EMG after spike-triggered averaging, called MSEMG-MUAP, correlated well (r = 0.82, P < 0.0001) with those of macro EMG. Moreover, the MSEMG-MUAP recording during a ramp force contraction exhibited the common size principle phenomenon during motor unit recruitment. The results of this study demonstrate that the MSEMG-MUAP measurement is a feasible approach for estimating the motor unit size from the skin surface.