Long-duration polyphasic motor unit potentials in myopathies: a quantitative study with pathological correlation

In most myopathies, the duration of motor unit action potentials (MUAPs) is shorter than normal. However, polyphasic MUAPs of duration longer than 20% of the control mean, (long-duration polyphasic potentials, or LDPPs) may be seen. We therefore analyzed the incidence and meaning of LDPPs using quantitative MUAP analysis in 41 patients with different myopathies. The mean duration of all potentials was reduced in only 64% of patients because LDPPs increased the mean. When only simple potentials were considered, however, the mean duration was decreased in 95% of patients. This observation confirms the need to exclude LDPPs when calculating the mean duration of MUAPs for diagnosis. We found LDPPs most often in chronic polymyositis and in one patient with Becker Muscular Dystrophy. LDPPs are attributed to desynchronization of single-fiber potentials within the MUAP and may be due to slow conduction in regenerating muscle fibers.     

Quantitative analysis of individual motor unit potentials: a proposition for standardized terminology and criteria for measurement

The physiology of the motor unit potential (MUP) is reviewed. The aim is to identify the electrophysiological events in the motor unit that generate the individual parts of the MUP. This is based on insight gained from new experimental techniques, such as single-fiber electromyography (EMG), scanning EMG, and simulation studies of the MUP. A terminology for the different parts of the MUP is also suggested, and nine parameters used to describe different features of the MUP are delineated: duration, spike duration, amplitude, area, spike area, phases, turns, satellites, and variability. Technical aspects, such as electrode type, filtering, and sampling rate of the computers, are discussed as well. In Appendix A, different manual and computer-aided methods for quantitative MUP analysis are described. Despite minor systematic differences between the methods, MUP durations measured by different methods correlate highly with each other (Appendix B). The manual and computer-aided methods have comparable variability between repeated measurements.     

Modeling studies on irregular motor unit potentials.

OBJECTIVE: The aim of these modeling studies on the generation of motor unit potentials (MUPs) was to establish the influence of MU parameters and recording conditions on the shape irregularity of MUPs. The focus was on the dependence of the irregularity of MUPs on fiber density, fiber diameters and the recording distance from the end-plate zone. METHODS: The study was performed using the 'EMG Simulator' software for modeling of MUP and our own software for calculations. RESULTS: The results indicate that the irregularity of a MUP increases with increased recording distance from the end-plate zone and decreases with increased fiber diameter and/or with increased fiber density. The quantitative relationship between these factors has been derived. CONCLUSIONS: The relationships determine the structural conditions in which irregular potential may be generated and therefore may be helpful in the interpretation of atypical MUPs.     

Decomposition-based quantitative electromyography: effect of force on motor unit potentials and motor unit number estimates

Decomposition-based quantitative electromyography (DQEMG) allows for the collection of motor unit potentials (MUPs) over a broad range of force levels. Given the size principle of motor unit recruitment, it may be necessary to control for force when using DQEMG for the purpose of deriving a motor unit number estimate (MUNE). Therefore, this study was performed to examine the effect of force on the physiological characteristics of concentric needle- and surface-detected MUPs and the subsequent impact on MUNEs obtained from the first dorsal interosseous (FDI) muscle sampled using DQEMG. Maximum M waves were elicited in 10 subjects with supramaximal stimulation of the ulnar nerve at the wrist. Intramuscular and surface-detected EMG signals were collected simultaneously during 30-s voluntary isometric contractions performed at specific percentages of maximal voluntary contraction (MVC). Decomposition algorithms were used to identify needle-detected MUPs and their individual MU firing times. These MU firing times were used as triggers to extract their corresponding surface-detected MUPs (S-MUPs) using spike-triggered averaging. A mean S-MUP was then calculated, the size of which was divided into the maximum M-wave size to derive a MUNE. Increased levels of contraction had a significant effect on needle- and surface-detected MUP size, firing rate, and MUNE. These results suggest that force level is an important factor to consider when performing quantitative EMG, including MUNEs with this method.     

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.     

Effect of small motor unit potentials on the motor unit number estimate

Small surface motor unit potentials (S-MUPs) may have a negative influence on the variability of the motor unit number estimate (MUNE). According to published consensus criteria S-MUPs with a negative peak amplitude smaller than 10 muV should be omitted. The effect of omitting small S-MUPs on the MUNE was evaluated using a simulation model. The model incorporated a healthy and amyotrophic lateral sclerosis (ALS) distribution formed with real S-MUPs. Using a random drawing process the MUNE was calculated with and without small S-MUPs. In the healthy population 27% of all S-MUPs were small. MUNE determined without these S-MUPs was marginally less variable. However, MUNE values dropped about 24% at a sample size of 20. In ALS, only 12% of the total population of 130 S-MUPs were small. MUNE dropped about 12% without the small S-MUPs. By omitting small S-MUPs the differences between the healthy and ALS distributions become smaller. Therefore, incorporating small S-MUPs in the estimate is suggested.     

Origin of surface motor unit potentials in hypothenar motor unit number estimation

Introduction: Far‐field potentials (FFPs) from muscles other than the abductor digiti minimi (ADM) may interfere with motor unit number estimation (MUNE) from that muscle. Methods: We identified the origin of each surface motor unit potential (SMUP) during hypothenar MUNE using the multiple point stimulation method in 20 control subjects by recording from individual ulnar‐innervated muscles with a common proximal reference (pref). Results: ADM SMUPs comprised 39.0% of the accepted SMUPs, followed by those from the fourth dorsal interosseous muscle (14.0%), the fourth lumbrical muscle (9.2%), and the second and third palmar interosseous muscles (8.8% each). The percentage of ADM SMUPs varied from 18% to 73% of accepted SMUPs among individual subjects. Accepted non‐ADM SMUPs were usually much smaller than ADM SMUPs, and many more non‐ADM SMUPs were excluded due to their small size. Conclusions: A large contribution from non‐ADM or non‐hypothenar SMUPs obscures the meaning of the MUNE value. Muscle Nerve, 48: 185–190, 2013     

Simulation of myopathic motor unit action potentials

Normal motor units (MUs) were simulated and their architecture altered to simulate the changes produced by myopathy. The concentric needle electromyographic recordings of motor unit action potentials (MUAPs) from the MUs were then also simulated. These simulated MUAPs showed features that are seen in myopathy: normal amplitude and slightly reduced area, MUAPs with simple waveform and reduced duration, and complex MUAPs with normal or increased duration. The MUAP waveforms were complex because of increased variability of fiber diameter and not because of loss of muscle fibers. The MUAP duration increased when the variability of fiber diameter increased. Finally, MUAPs similar to those seen in neurogenic diseases were produced from MUs in which the only abnormality was increased variability of fiber diameter.     

Effectiveness of motor unit potentials classification using various parameters and indexes.

OBJECTIVES: The aim of this study was to compare the effectiveness of quantities such as amplitude, duration, area, size index, number of peaks and irregularity coefficient applied to the classification of motor unit action potentials (MUAPs) in neuromuscular disorders. METHODS: We have analyzed 215 potentials recorded from 20 neurogenic patients and 240 potentials recorded from 14 myogenic patients. The statistical values for each parameter and correlation coefficients between parameters have been analyzed. RESULTS: The percentages of potentials unclassified by amplitude, duration, area/amplitude, area and size index were 31, 34, 28, 15 and 15%, respectively. Neither the number of phases and turns nor the irregularity coefficient may be used for the differentiation between neuro- and myogenic groups. There was also a set of MUAPs that were not properly classified by any of the above parameters. Among the unclassified potentials there were more potentials from myogenic cases. These unclassified myogenic MUAPs were predominantly irregular, whereas most of the unclassified neurogenic MUAPs were simple. In neurogenic cases, parameters describing the size of the potential (amplitude, duration, area) were not significantly correlated with those describing the shape (number of phases and turns), whereas in myogenic cases some correlation between these parameters was significant. CONCLUSIONS: MUAP quantities, due to the different correlation between them in myogenic and neurogenic disorders, have different sensitivities. Indexes were more sensitive than simple parameters and were also much more effective in the evaluation of atypical potentials.     

Fatigue-related changes in motor unit action potentials of adult cats.

The purpose of this study was to quantify the changes in motor-unit action potentials (MUAP) and force during a standard motor-unit fatigue test. MUAP waveforms were characterized by the measurement of amplitude, duration, area, and shape (as reflected in a coefficient of proportionality). Fatigue-resistant motor units exhibited small, but statistically significant, changes in MUAP amplitude and area during the fatigue test, whereas fatigable motor units displayed variable changes in MUAP amplitude, duration, and area. For all motor-unit types, the coefficient of proportionality did not change, and hence the change in MUAP area was proportional to the combined changes in amplitude and duration. The between- and within-train changes in MUAP were also distinct for the fatigue-resistant and fatigable motor units. Although several mechanisms could be responsible for the changes in the MUAP as the fatigue test proceeded, the dissociation of the time courses for MUAP and force indicated that these MUAP changes were not the principal reason for the decline in force under these conditions.     

The diagnostic reliability of magnetically evoked motor potentials in multiple sclerosis.

In a prospective study, we evaluated the technique of magnetically evoked motor potentials (MEP) in the diagnosis of multiple sclerosis (MS). We consecutively included 68 patients with symptoms or signs compatible with a demyelinative CNS affection. We subjected all patients to CSF analysis, MRI studies of the brain and brainstem, visual evoked potentials (VEP), brainstem auditory evoked potentials (BAEP), and somatosensory evoked potentials (SSEP). We then used the results to categorize the patients according to the Poser criteria of multiple sclerosis. Blinded from the results of the above investigations, one of the authors made MEP recordings from three muscles in the upper limbs and two in the lower limbs in all 68 patients. Forty patients received an MS diagnosis, and in these, MRI was positive in 88%, MEP in 83%, VEP in 67%, SSEP in 63%, and BAEP in 42%. As to the diagnosis of MS, the reliability of a prolonged central motor conduction time (CMCT) was 0.83 (0.73 to 0.93), while the reliability of a normal CMCT was 0.75 (0.61 to 0.98). The information gained by MRI was best supplemented by VEP. Of the neurophysiologic tests, the MEP was in closest agreement with the MRI with a concordance of 85%.     

Within-subject reliability of motor unit number estimates and quantitative motor unit analysis in a distal and proximal upper limb muscle.

OBJECTIVE: To establish within-subject reliability of motor unit number estimates (MUNEs) and quantitative MU analysis using decomposition-based quantitative electromyography (DQEMG). METHODS: Following the acquisition of a maximum M-wave, needle and surface-detected EMG signals were collected during contractions of the first dorsal interrosseous (FDI) and biceps brachii (BB). DQEMG was used to extract motor unit potential (MUP) trains and surface-detected MUPs associated with each train, the mean size of which was divided into the maximum M-wave to obtain a MUNE. Retests were performed following the initial test to evaluate reliability. RESULTS: Subjects test-retest MUNEs were highly correlated (r=0.72 FDI; 0.97 BB) with no significant differences between test and retest MUNE values (P>0.10). Ninety-five percent confidence intervals were calculated to establish the range of expected retest MUNE variability and were +/-41 MUs for the FDI and BB. Quantitative information pertaining to MU size, complexity and firing rate were similar for both tests. CONCLUSION: MUNEs and quantitative MU data can be obtained reliably from the BB and FDI using DQEMG in individual subjects. SIGNIFICANCE: Establishing within-subject reliability of MUNEs and quantitative MU analysis allow clinicians to longitudinally follow changes in the MU pool of individuals with disorders of the central or peripheral nervous system in addition to assessing their response to treatments.     

Satellite potentials of motor unit action potentials in normal muscles: a new hypothesis for their origin.

OBJECTIVE: A satellite potential is a late component of the motor unit action potential (MUAP) that occurs both in pathologic and normal muscle. We investigated the physiological mechanisms responsible for satellite potentials in normal muscle by relating the latencies of MUAP features to the timing of the underlying electrical events. METHODS: We analyzed 21 MUAPs with satellite potentials that had been recorded using a monopolar needle electrode from brachial biceps and tibialis anterior muscles in 10 normal subjects. We estimated the endplate-to-electrode, endplate-to-tendon, and satellite propagation times from the latencies, with respect to the MUAP onset, of the MUAP spike, terminal wave, and satellite. RESULTS: Satellite latencies ranged from 8.8 to 32 ms, too long to be explained by mechanisms involving regenerating axons or atrophic muscle fibers. The spike-to-satellite time intervals approximated either twice the spike-to-terminal-wave interval (17 MUAPs) or twice the terminal-wave latency (4 MUAPs). CONCLUSIONS: These results are consistent with the hypothesis that satellite potentials are due to retrograde propagation in a non-innervated muscle fiber that is connected with an innervated muscle fiber at one of the muscle/tendon junctions. Such a configuration could arise as a result of longitudinal muscle-fiber splitting.     

Spontaneous rhythmic motor unit potentials in the carpal tunnel syndrome.

In 18 out of 164 consecutive patients with carpal tunnel syndrome ongoing spontaneous rhythmic motor unit potentials were found in the lateral thenar muscles. The frequency ranged from 3 to 9 Hz with an average of 6 Hz. The spontaneous rhythmic motor unit potentials appeared to be doublets in many cases. Ischaemia usually caused a marked increase of spontaneous activity within 1 to 2 minutes. Directly after ischaemia the spontaneous rhythmic motor unit potentials subsided for some time. Variable periods of suppression also occurred after forceful muscle contraction and during repetitive supramaximal stimulation of the median nerve. In four patients where the median nerve had been blocked at the level of the elbow with lidocaine the spontaneous activity remained, indicating that the site of origin was distal, probably at the compression site at the carpus.     

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.     

Inter-rater reliability of motor unit number estimates and quantitative motor unit analysis in the tibialis anterior muscle

ObjectiveTo establish the inter-rater reliability of decomposition-based quantitative electromyography (DQEMG) derived motor unit number estimates (MUNEs) and quantitative motor unit (MU) analysis.MethodsUsing DQEMG, two examiners independently obtained a sample of needle and surface-detected motor unit potentials (MUPs) from the tibialis anterior muscle from 10 subjects. Coupled with a maximal M wave, surface-detected MUPs were used to derive a MUNE for each subject and each examiner. Additionally, size-related parameters of the individual MUs were obtained following quantitative MUP analysis.ResultsTest–retest MUNE values were similar with high reliability observed between examiners (ICC = 0.87). Additionally, MUNE variability from test–retest as quantified by a 95% confidence interval was relatively low ($\pm 28$ MUs). Lastly, quantitative data pertaining to MU size, complexity and firing rate were similar between examiners.ConclusionMUNEs and quantitative MU data can be obtained with high reliability by two independent examiners using DQEMG.SignificanceEstablishing the inter-rater reliability of MUNEs and quantitative MU analysis using DQEMG is central to the clinical applicability of the technique. In addition to assessing response to treatments over time, multiple clinicians may be involved in the longitudinal assessment of the MU pool of individuals with disorders of the central or peripheral nervous system.     

Diagnostic yield of the analysis of the pattern of electrical activity of muscle and of individual motor unit potentials in neurogenic involvement.

The pattern of electrical activity and the properties of individual motor unit potentials were analysed in the flexor muscles of the forearm of 15 patients with motor neurone disease and 15 patients with a lesion of the brachial plexus. The best diagnostic yield from the pattern of electrical activity was obtained when the force was 30% of maximum: The number of spikes (turns/15 s) was diminished in 70% of the patients; none showed the increase in turns characteristic of myopathy. The decrease in the number of turns was often associated with an increased incidence of long time intervals between turns and with an increased amplitude between turns. The mean duration of individual motor unit potentials was prolonged in 78% of the patients; none showed the decrease in mean duration characteristic of myopathy. Prolongation of the mean duration of motor unit potentials produced by cooling of normal muscle, led to a decrease in the number of turns. This suggested that the diminished number of turns in neurogenic involvement was due mainly to the prolonged duration of motor unit potentials.