Quantitative Motor Unit Potential Analysis Research Articles

Increased prevalence of mild myopathic changes in the post-COVID-19 duration

ABSTRACT Objective There are reports of peripheral nerve and muscle involvement during or after coronavirus disease 2019 (COVID-19), even following a mild infection. Here, we aimed to analyze the objective findings regarding peripheral nerve, neuromuscular junction, and muscle function using electrophysiology in patients with a previous COVID-19 infection. Methods All consecutive patients with a history of COVID-19 were questioned for post-COVID-19 duration-related neurological complaints via Composite Autonomic Symptom Score-31 (COMPASS-31), modified Toronto Neuropathy score (mTORONTO), and Fatigue Severity Scale (FSS). Patients were dichotomized into two groups based on their scores in the questionnaire. Group 1 (patients with high scores in any area of the questionnaire) and Group 2 (patients with normal scores in all sections of the questionnaire). In the second step, Group 1 was invited to a preplanned hospital visit for electrophysiological analysis, including nerve conduction studies, repetitive nerve stimulation, needle electromyography (EMG), quantitative motor unit potential analysis (qMUP), and single fiber EMG. We included 106 patients in the study. According to the questionnaire, 38 patients constituted Group 1, and 68 formed Group 2. Results Of the 38 patients, 14 accepted and underwent preplanned electrophysiological examinations. Needle EMG revealed small, short, polyphasic MUPs with early recruitment, and qMUP analysis demonstrated an increased percentage of polyphasic potentials in three patients. The examinations in other patients were unremarkable. Conclusions The high prevalence of complaints and objective myopathic findings in our cohort implicated the role of muscle involvement in the post-COVID-19 duration. Considering the socioeconomic and psychological burden of the post-COVID-19 duration among individuals and societies, a better understanding of the symptoms and myopathy is warranted.

Quantitative motor unit potential analysis and nerve conduction studies for detection of subclinical peripheral nerve dysfunction in patients with compensated liver cirrhosis

BackgroundPeripheral neuropathy is an underestimated problem of compensated liver cirrhosis despite its negative effect on quality of life. The aim was to assess the role of meticulous electrophysiological screening (nerve conduction responses and quantitative motor unit potential analysis) of subclinical peripheral nerve dysfunction in patients with compensated cirrhosis and also to explore its relations with different characteristics of liver disease. Severity of cirrhosis was assessed by Child–Pugh and albumin-bilirubin (ALBI) scores.ResultsPrevalence of hepatic neuropathy (HN) was 82%. It involved sensory and motor fibers (66%), and its pathophysiology was axonal (53.7%) or mixed axonal and demyelinating (46.3). The most sensitive discriminating tests were ulnar sensory conduction velocity (area under curve (AUC) = 0.915) and peak latency (AUC = 0.887), peroneal motor conduction velocity (AUC = 0.885), ulnar distal motor latency (AUC = 0.842), and first dorsal interosseous number of phases (AUC = 0.736). HN showed significant correlation with the severity of liver disease assessed by both child (P = 0.029) and ALBI (P = 0.016) scores and also correlated with the low serum albumin level (P = 0.001).ConclusionsSubclinical mild axonal polyneuropathy is very common in post-hepatitis C compensated cirrhosis picked up by meticulous electrophysiological testing, and it is related to severity of liver cirrhosis and low serum albumin level.

S130. Muscle velocity recovery cycles in intensive care unit patients

Introduction Critical illness myopathy is a disabling condition that develops in critically ill patients and presents as diffuse muscle weakness and failure to wean from mechanical ventilation. Muscle Velocity Recovery Cycles (MVRC) enable in vivo assessment of muscle membrane properties. We aimed in this study to examine whether MVRC may detect changes in patients with delayed wean from mechanical ventilation. Methods Six patients (mean age: 52.4 ± 5.8) with failure to wean from mechanical ventilation and 24 healthy controls (mean age: 53.2 ± 3.2) were prospectively included. In addition to conventional nerve conduction studies (NCS) and electromyography, all patients were examined by MVRC in the anterior tibial muscle using Qtrac-software. MVRC parameters consisted of muscle relative refractory period (MRRP) and early and late supernormality after 1, 2 or 5 conditioning stimuli. Results Motor NCS of peroneal and tibial and sensory NCS of sural nerves were normal. None of the patients had spontaneous activity and quantitative motor unit potential analysis was normal. MRRP was significantly prolonged in patients (5.9 ± 0.7) compared to healthy controls (3.6 ± 0.09), and early supernormality was lower in patients than healthy controls after 2 (5.1 ± 1.1 vs 11.3 ± 0.4) and 5 (4.6 ± 1.1 vs 13.5 ± 0.4) conditioning stimuli. Similarly, late supernormality was decreased in patients compared to healthy controls after 1 (2.4 ± 0.5 vs 3.9 ± 0.2), 2 (1.4 ± 0.4 vs 2.8 ± 0.1) and 5 (3.1 ± 0.8 vs 7.9 ± 0.2) conditioning stimuli (t test, p Conclusion Changes in MVRC in patients with failure to wean from mechanical ventilation may suggest critical illness myopathy in these patients. MVRC may detect changes in the muscle membrane before spontaneous activity and motor unit potential changes are seen. Future studies with larger patient groups and follow-up examinations are needed.

The utility of motor unit number estimation methods versus quantitative motor unit potential analysis in diagnosis of ALS

ObjectiveTo compare the diagnostic utility of motor unit number estimation (MUNE) methods to motor unit potential (MUP) analysis in amyotrophic lateral sclerosis (ALS). MethodsTwenty-five patients (1 definite, 11 probable, 9 possible ALS and 4 progressive muscular atrophy) and 22 healthy controls were prospectively included. Quantitative MUP analysis and three MUNE methods; Multiple Point Stimulation MUNE (MPS), Motor Unit Number Index (MUNIX) and MScanFit MUNE (MScan) were done in abductor pollicis brevis muscle. The sensitivities were compared by McNemar chi-square test. MUNE, MUP and revised ALS Functional Rating Scale (ALSFRS-R) parameters were correlated by regression analysis. ResultsThe sensitivities of MPS (76%) and MScan (68%) were higher than MUP duration (36%) and amplitude (40%) in detecting motor unit loss (p < 0.05). MUNE methods increased the categorical probability from possible to probable ALS in 4 patients (16%). There was only significant correlation between ALSFRS-R and MScan (r = 0.443, p = 0.027) among the electrophysiological tests. MUNE methods did not correlate to MUP parameters. ConclusionsMUNE methods are more sensitive in showing abnormality than MUP analysis. SignificanceMUNE methods, in particular MScan, may have the potential to be implemented in the clinical practice for diagnosis and follow-up of neuromuscular disorders particularly ALS.

Template-operated MUP analysis is not accurate in the diagnosis of myopathic or neuropathic changes in the diaphragm

The aim of this study was to evaluate the quantitative motor unit potential (MUP) analysis in the diagnosis of myopathy and neuropathy of the diaphragm. Diaphragm template-operated quantitative EMG were performed in 30 patients with myotonic dystrophy type 1 (DM1), 17 with myotonic dystrophy type 2 (DM2) and 40 with amyotrophic lateral sclerosis (ALS). Low MUP amplitude precluded MUP analysis in 21% of DM1 patients. Only a single DM1 patient had EMG findings consistent with myopathy. In this patient, and another 4 DM1 and 3 DM2 patients, findings were consistent with neuropathy. Neuropathic MUP changes were found in 92% of ALS patients, but due to motor neuron cell loss in only 60% MUPs analyses could be done. Phrenic nerve conduction studies correlated with respiratory function tests, whereas MUP parameters did not. Quantitative MUP analysis was not able to adequately sample diaphragm MUPs in patients with chronic myopathy or motor neuronopathy.

O25 Motor unit number estimation methods compared to quantitative motor unit potential analysis in amyotrophic lateral sclerosis

Objectives To compare the sensitivity of MUNE methods to quantitative motor unit potential (MUP) analysis in amyotrophic lateral sclerosis (ALS). Methods Twenty-four patients (1 definite, 11 probable and 8 possible ALS according to awaji criteria as reference standard, and 4 progressive muscular atrophy) were included. Patients were evaluated by revised ALS functional rating scale (ALSFRS-R). In all patients, quantitative MUP analysis and three MUNE methods; MScanFit MUNA (MScan), multiple point stimulation MUNE (MPS), and Motor Unit Number Index (MUNIX) were done in abductor pollicis brevis (APB). MUNE results were compared to 20 age-matched healthy controls and EMG to laboratory controls. The sensitivity of each methods was compared using McNemar chi-square test. MUNE values and MUP parameters were correlated to ALSFRS-R by regression analysis. Results MScan (70%) and MPS (61%) more frequently showed abnormality than MUP duration (35%) whereas only MScan was more often abnormal than MUP amplitude (39%) ( p r = 0.42, p = 0.03) but not MPS ( r = 31), MUNIX ( r = 35), MUP duration ( r = 0.19) or amplitude ( r = 0.09). Discussion MUP analysis gives information about reinnervation, but not about loss of motor units. Therefore MUNE methods may be preferable in ALS electrodiagnosis. Conclusions MUNE methods, particularly MScan, had higher sensitivities than MUP analysis in detection of abnormality in APB and were correlated better to ALSFRS-R. Significance MScan is a promising novel method which should be considered in diagnosis and monitoring disease progression in ALS.

Lower motor neuron dysfunction in ALS.

In the motor system there is a complex interplay between cortical structures and spinal cord lower motor neurons (LMN). In this system both inhibitory and excitatory neurons have relevant roles. LMN loss is a marker of motor neuron disease/amyotrophic lateral sclerosis (MND/ALS). Conventional needle electromyography (EMG) does not allow LMN loss to be quantified. Measurement of compound muscle action potential (CMAP) amplitude or area, and the neurophysiological index, provide a surrogate estimate of the number of functional motor units. Increased motor neuronal excitability is a neurophysiological marker of ALS in the context of a suspected clinical and electrophysiological diagnosis. In the LMN system, fasciculation potentials (FPs) are the earliest changes observed in affected muscles, a feature of LMN hyperexcitability. Reinnervation is best investigated by needle EMG although other methods can be explored. Moreover needle EMG give information about the temporal profile of the reinnervation process, important ancillary data. Quantitative motor unit potential analysis is a valuable method of evaluating reinnervation. The importance of FPs has been recognized in the Awaji criteria for the electrodiagnosis of ALS, criteria that are a sensitive adjunct to the revised El Escorial criteria. Finally, functionality of LMN's, and perhaps excitability studies in motor nerves, aids understanding of the disease process, allowing measurement of potential treatment effects in clinical trials. Other investigational techniques, such as electrical impedance myography, muscle and nerve ultrasound, and spinal cord imaging methods may prove useful in future.

Intra-rater reliability of motor unit number estimation and quantitative motor unit analysis in subjects with amyotrophic lateral sclerosis

ObjectivesTo assess the intra-rater reliability of decomposition-enhanced spike-triggered averaging (DE-STA) motor unit number estimation (MUNE) and quantitative motor unit potential analysis in the upper trapezius (UT) and biceps brachii (BB) of subjects with amyotrophic lateral sclerosis (ALS) and to compare the results from the UT to control data. MethodsPatients diagnosed with clinically probable or definite ALS completed the experimental protocol twice with the same evaluator for the UT (n=10) and BB (n=9). ResultsIntra-rater reliability for the UT was good for the maximum compound muscle action potential (CMAP) (ICC=0.88), mean surface-detected motor unit potential (S-MUP) (ICC=0.87) and MUNE (ICC=0.88), and for the BB was moderate for maximum CMAP (ICC=0.61), and excellent for mean S-MUP (ICC=0.94) and MUNE (ICC=0.93). A significant difference between tests was found for UT MUNE. Comparing subjects with ALS to control subjects, UT maximum CMAP (p<0.01) and MUNE (p<0.001) values were significantly lower, and mean S-MUP values significantly greater (p<0.05) in subjects with ALS. ConclusionsThis study has demonstrated the ability of the DE-STA MUNE technique to collect highly reliable data from two separate muscle groups and to detect the underlying pathophysiology of the disease. SignificanceThis was the first study to examine the reliability of this technique in subjects with ALS, and demonstrates its potential for future use as an outcome measure in ALS clinical trials and studies of ALS disease severity and natural history.

Sacral neurophysiologic study in patients with chronic spinal cord injury

Neurophysiologic testing of the sacral reflex has demonstrated utility in the diagnosis of sacral lower motor neuron lesions. The aim of the present study was to also apply this test to patients with upper motor neuron lesions. A group of 16 male patients with chronic suprasacral spinal cord lesions was prospectively recruited. In addition to history and clinical neurologic examination (including anal sphincter tone, saddle sensation, and penilo-cavernosus reflex testing), sacral neurophysiologic studies were performed. Neurophysiologic testing included quantitative electromyography of the external anal sphincter (motor unit potential (MUP) count during relaxation and MUP analysis), and neurophysiologic measurement of the penilo-cavernosus reflex (elicitation threshold and latency) on electrical stimulation. The findings were compared to data obtained in the control group of 26 men. Clinical elicitability of the penilo-cavernosus reflex was increased, and the neurophysiologically measured reflex threshold reduced in patients (<0.02). No significant differences were found in clinical assessment of anal sphincter tone, MUP count, reflex latency, and quantitative MUP analysis. The diagnostic sensitivity of individual parameters was low (≤25%). In patients with chronic spinal cord injury, increased elicitability of the penilo-cavernosus reflex was found. However, none of the assessed neurophysiologic parameters was found to assist in the diagnosis of the sacral upper motor neuron lesion in individual patients. The shortened sacral reflex latency found in individual patients is therefore not a consequence of a suprasegmental lesion, but rather of the low position of the conus medullaris (e.g., in tethered cord syndrome).

Quantitative motor unit potential analysis in the diaphragm: A normative study

Although quantitative motor unit potential (MUP) analysis has an established role in other skeletal muscles, it has not been performed in the diaphragm. The aim of the present study was to test whether such studies are possible and to establish normative data. Twenty-nine healthy volunteers (15 men), aged 21-65 years (median, 33 years), were studied using standard concentric needle electrodes, and equipment with the facility for template-operated multi-MUP analysis. Needle electrodes were inserted into the right medial recess of the seventh to ninth intercostal spaces. During MUP sampling, subjects were asked to hold their breath in partial inspiration for 5 s. At least 20 MUPs were obtained in 28 subjects. Diaphragmatic MUPs were confirmed to be much smaller than those of limb muscles. We found diaphragmatic quantitative MUP analysis to be possible in healthy volunteers. However, further studies in patient groups are needed to establish the feasibility and clinical value of such studies.

The diagnostic power of motor unit potential analysis: an objective bayesian approach.

The notion of a "myopathic" or "neuropathic" electromyogram (EMG) is usually based on qualitative visual and acoustical impressions. Conventional quantification defines abnormality but not diagnosis, which requires interpretation of patterns of change. Discriminant analysis is a model for this multivariate decision. It tells how probable it is that a motor unit potential (MUP) comes from a normal, myopathic, or neuropathic muscle. Accumulation of single MUP information by a sequential Bayesian algorithm produced diagnostic probabilities above 0.95 in 91% of all muscles (223 biceps brachii muscles from 80 patients with motoneuron disorders, 56 patients with neuropathies, 71 patients with myopathies, and 34 controls). Two muscles from patients with neurogenic disorders were misclassified as "myopathic." Misclassification was more frequent only in myositis (4 of 28 muscles) and in oculopharyngeal muscular dystrophy (2 of 4 muscles). MUP discriminant classification was as sensitive as, and more specific than, conventional quantitative EMG, which discriminated between myopathic and neuropathic in only 22% of the muscles. This rate was 59% for discriminant analysis. As a knowledge-based expert system, MUP discriminant analysis successfully distinguishes between myopathic, neuropathic, and unclassifiable MUP samples. It discloses more information than conventional quantitative MUP analysis.

Evoked fasciculations in patients with amyotrophic lateral sclerosis

In the motor system there is a complex interplay between cortical structures and spinal cord lower motor neurons (LMN). In this system both inhibitory and excitatory neurons have relevant roles. LMN loss is a marker of motor neuron disease/amyotrophic lateral sclerosis (MND/ALS). Conventional needle electromyography (EMG) does not allow LMN loss to be quantified. Measurement of compound muscle action potential (CMAP) amplitude or area, and the neurophysiological index, provide a surrogate estimate of the number of functional motor units. Increased motor neuronal excitability is a neurophysiological marker of ALS in the context of a suspected clinical and electrophysiological diagnosis. In the LMN system, fasciculation potentials (FPs) are the earliest changes observed in affected muscles, a feature of LMN hyperexcitability. Reinnervation is best investigated by needle EMG although other methods can be explored. Moreover needle EMG give information about the temporal profile of the reinnervation process, important ancillary data. Quantitative motor unit potential analysis is a valuable method of evaluating reinnervation. The importance of FPs has been recognized in the Awaji criteria for the electrodiagnosis of ALS, criteria that are a sensitive adjunct to the revised El Escorial criteria. Finally, functionality of LMN’s, and perhaps excitability studies in motor nerves, aids understanding of the disease process, allowing measurement of potential treatment effects in clinical trials. Other investigational techniques, such as electrical impedance myography, muscle and nerve ultrasound, and spinal cord imaging methods may prove useful in future.

Postpolio muscular atrophy. Diagnostic utility of macroelectromyography.

Muscles recovered from previous poliomyelitis may exhibit new weakness and wasting years after the original infection (postpolio muscular atrophy). Routine electrophysiological and morphological techniques may not differentiate weakening muscles from previously affected but stable muscles. We used quantitative motor unit potential analysis, single-fiber electromyography, and macroelectromyography (macro-EMG) to determine if these techniques could identify weakening muscles. We classified 18 previously affected muscles according to strength from 12 patients who had had poliomyelitis 18 to 50 years earlier. Muscles of normal strength had large-amplitude macro-EMG signals (1000 to 4110 microV; normal level, less than 390 microV). Muscles with new weakness and atrophy had smaller than expected macro-EMG amplitude (130 to 450 microV). Two muscles with weakness and atrophy unchanged since the original childhood infection had markedly increased macro-EMG amplitude (1710 and 1860 microV), suggesting the increased macro-EMG amplitude is due to stable reinnervation not strength. Mean motor unit potential duration and amplitude were lower in newly weakened muscles, but jitter, fiber density, and blocking were similar in all muscles regardless of strength. We conclude that low-amplitude macro-EMG signals may be useful in the identification of muscles weakened by postpolio muscular atrophy.

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.