Behaviour of motor unit action potential rate, estimated from surface EMG, as a measure of muscle activation level

Background  

Surface electromyography (EMG) parameters such as root-mean-square value (RMS) are commonly used to assess the muscle activation level that is imposed by the central nervous system (CNS). However, RMS is influenced not only by motor control aspects, but also by peripheral properties of the muscle and recording setup. To assess motor control separately, the number of motor unit action potentials (MUAPs) per second, or MUAP Rate (MR) is a potentially useful measure. MR is the sum of the firing rates of the contributing MUs and as such reflects the two parameters that the CNS uses for motor control: number of MUs and firing rate.     

Motor unit potential morphology differences in individuals with non-specific arm pain and lateral epicondylitis

Background

The pathophysiology of non-specific arm pain (NSAP) is unclear and the diagnosis is made by excluding other specific upper limb pathologies, such as lateral epicondylitis or cervical radiculopathy. The purpose of this study was to determine: (i) if the quantitative parameters related to motor unit potential morphology and/or motor unit firing patterns derived from electromyographic (EMG) signals detected from an affected muscle of patients with NSAP are different from those detected in the same muscle of individuals with lateral epicondylitis (LE) and/or control subjects and (ii) if the quantitative EMG parameters suggest that the underlying pathophysiology in NSAP is either myopathic or neuropathic in nature.

Methods

Sixteen subjects with NSAP, 11 subjects with LE, eight subjects deemed to be at-risk for developing a repetitive strain injury, and 37 control subjects participated. A quantitative electromyography evaluation was completed using decomposition-based quantitative electromyography (DQEMG). Needle- and surface-detected EMG signals were collected during low-level isometric contractions of the extensor carpi radialis brevis (ECRB) muscle. DQEMG was used to extract needle-detected motor unit potential trains (MUPTs), and needle-detected motor unit potential (MUP) and surface detected motor unit potential (SMUP) morphology and motor unit (MU) firing rates were compared among the four groups using one-way analysis of variance (ANOVA). Post hoc analyses were performed using Tukey's pairwise comparisons.

Results

Significant group differences were found for all MUP variables and for MU firing rate (p < 0.006). The post-hoc analyses revealed that patients with NSAP had smaller MUP amplitude and SMUP amplitude and area compared to the control and LE groups (p < 0.006). MUP duration and AAR values were significantly larger in the NSAP, LE and at-risk groups compared to the control group (p < 0.006); while MUP amplitude, duration and AAR values were smaller in the NSAP compared to the LE group. SMUP duration was significantly shorter in the NSAP group compared to the control group (p < 0.006). NSAP, LE and at-risk subjects had lower mean MU firing rates than the control subjects (p < 0.006).

Conclusion

The size-related parameters suggest that the NSAP group had significantly smaller MUPs and SMUPs than the control and LE subjects. Smaller MUPs and SMUPs may be indicative of muscle fiber atrophy and/or loss. A prospective study is needed to confirm any causal relationship between smaller MUPs and SMUPs and NSAP as found in this work.     

Acute effects of bi-hemispheric transcranial direct current stimulation on the neuromuscular function of patients with chronic stroke: A randomized controlled study

BackgroundMuscle weakness in patients with chronic stroke is due to neuromuscular disorders such as muscle atrophy, loss of voluntary activation or weak muscle contractile properties which are majored by the imbalance of interhemispheric inhibition following stroke. In patients with chronic stroke, unilateral transcranial direct current stimulation improved the maximal isometric strength of paretic knee extensors, but bilateral transcranial direct current stimulation failed to improve concentric strength. This study aimed to assess if a bilateral current stimulation improves isometric maximal strength, voluntary activation and contractile properties of knee extensors in patients with chronic stroke.MethodsThirteen patients with chronic stroke and eight young healthy individuals participated in this randomized, simple-blinded, crossover study that included two experimental sessions: one with sham bilateral transcranial direct current stimulation and another with effective bilateral transcranial direct current stimulation (20 min, 2 mA). In the stroke patients, the anode was placed over the primary motor cortex of the affected hemisphere and the cathode over the contralateral primary motor cortex. In healthy participants, the brain side targeted by the anode and the cathode was randomly assigned. In each session, participants performed three assessments of strength, voluntary activation and contractile properties: before, during and after effective/sham bilateral transcranial direct current stimulation.FindingsBilateral transcranial direct current stimulation had no effect on any neuromuscular assessments in both groups (All P values > 0.05, partial eta-squares varied from 0.02 to 0.06).InterpretationA single session of bilateral transcranial direct current stimulation did not compensate muscular weakness of knee extensors in patients with chronic stroke.     

Neural adjustment in the activation of the lower leg muscles through daily physical exercises in community-based elderly persons

Reflecting the rapidly aging population, community-based interventions in the form of physical exercise have been introduced to promote the health of elderly persons. Many investigation studies have focused on muscle strength in the lower leg as a potent indicator of the effect of physical exercises. The objective of this study was to assess the effect of long-term daily exercises on neural command in lower leg muscle activations. Twenty-six community-based elderly persons (13 men and 13 women; 69.8 +/- 0.5 years old) participated in this study. Daily exercise was comprised of walking for more than 30 min, stretching, muscle strengthening and balance exercise, and was continued for three months. Muscle strength and surface electromyography of the tibia anterior, rectus femoris, and biceps femoris were measured in maximum isometric voluntary contraction both before and after the intervention. The mean frequency of the firing of motor units was calculated based on fast Fourier transformation of the electromyography. As the results of the intervention, muscle strength increased significantly only in biceps femoris, whereas the mean frequency of motor units decreased significantly in every muscle, indicating that motor unit firing in lower frequency efficiently induces the same or greater strength compared with before the intervention. Thus, synchronization of motor units compensates for the lower frequency of motor unit firing to maintain muscular strength. In conclusion, long-term physical exercises in the elderly can modulate the neural adjustment of lower leg muscles to promote efficient output of muscle strength.     

Motor unit number estimation in the assessment of performance and function in motor neuron disease

Motor unit number estimation (MUNE) is a unique electrophysiologic test used to estimate the number of surviving motor units in a muscle or group of muscles. It is used most frequently to monitor lower motor neuron loss in amyotrophic lateral sclerosis and spinal muscle atrophy. Of particular interest is its use as an endpoint measure in clinical trials for these diseases. This article describes the principles of MUNE and the factors that need to be considered, and reviews several techniques that have been used in clinical trials and in monitoring progression. It then reviews experience with MUNE in clinical trials for amyotrophic lateral sclerosis and spinal muscle atrophy and discusses how MUNE correlates with measures of function.     

Fatigue effects on motor unit activity during submaximal contractions

OBJECTIVE: To examine motor unit changes during the development of fatigue in healthy subjects. DESIGN: Automated decomposition-enhanced spike-triggered averaging was used to characterize motor unit size and firing rate in the dominant vastus medialis during maintained contractions at 10% and 30% of maxima voluntary contraction (MVC). SETTING: Academic outpatient neuromuscular clinic. PARTICIPANTS: Healthy laboratory personnel. MAIN OUTCOME MEASURES: Surface electromyogram, surface-detected motor unit action potential amplitude (S-MUAP), mean firing rate, force (MVC), motor unit index. RESULTS: Surface electromyogram values and S-MUAP amplitudes increased during both 10% and 30% MVC fatiguing contractions, while mean firing rates decreased. A motor unit index, indicating the degree of motor unit pool activation, increased similarly to S-MUAP size, implying that new and larger units were recruited to maintain the contraction. Repeated contractions led to earlier motor unit changes and fatigue. CONCLUSION: During submaximal fatiguing contractions, additional motor units are activated to maintain strength. These changes begin early, within the first minute, particularly after a previous fatiguing effort.     

Intraoperative Monitoring of Segmental Spinal Nerve Root Function with Free-Run and Electrically-Triggered Electromyography and Spinal Cord Function with Reflexes and F-Responses

Background Context. Orthodromic ascending somatosensory evoked potentials and antidromic descending neurogenic somatosensory evoked potentials monitor spinal cord sensory function. Transcranial motor stimulation monitors spinal cord motor function but only activates 4–5% of the motor units innervating a muscle. Therefore, 95–96% of the motor spinal cord systems activating the motor units are not monitored. To provide more comprehensive monitoring, 11 techniques have been developed to monitor motor nerve root and spinal cord motor function. These techniques include: 1. neuromuscular junction monitoring, 2. recording free-run electromyography (EMG) for monitoring segmental spinal nerve root function, 3. electrical stimulation to help determine the correct placement of pedicle screws, 4. electrical impedance testing to help determine the correct placement of pedicle screws, 5. electrical stimulation of motor spinal nerve roots, 6. electrical stimulation to help determine the correct placement of iliosacral screws, 7. recording H-reflexes, 8. recording F-responses, 9. recording the sacral reflex, 10. recording intralimb and interlimb reflexes and 11. recording monosynaptic and polysynaptic reflexes during dorsal root rhizotomy. Objective. This paper is the position statement of the American Society of Neurophysiological Monitoring. It is the practice guideline for the intraoperative use of these 11 techniques. Methods. This statement is based on information presented at scientific meetings, published in the current scientific and clinical literature, and presented in previously-published guidelines and position statements of various clinical societies. Results. These 11 techniques when used in conjunction with somatosensory and transcranial motor evoked potentials provide a multiple-systems approach to spinal cord and nerve root monitoring. Conclusions. The techniques reviewed in this paper may be helpful to those wishing to incorporate these techniques into their monitoring program.     

Musculoskeletal complications of neuromuscular disease in children

A wide variety of neuromuscular diseases affect children, including central nervous system disorders such as cerebral palsy and spinal cord injury; motor neuron disorders such as spinal muscular atrophy; peripheral nerve disorders such as Charcot-Marie-Tooth disease; neuromuscular junction disorders such as congenital myasthenia gravis; and muscle fiber disorders such as Duchenne's muscular dystrophy. Although the origins and clinical syndromes vary significantly, outcomes related to musculoskeletal complications are often shared. The most frequently encountered musculoskeletal complications of neuromuscular disorders in children are scoliosis, bony rotational deformities, and hip dysplasia. Management is often challenging to those who work with children who have neuromuscular disorders.     

tDCS and motor training in individuals with central nervous system disease: A systematic review

BackgroundTranscranial direct current stimulation (tDCS) is a promising tool for patients with neurological disorders, as it increases cortical excitability, motor learning and functionality. The studies up to date have focused on the tDCS parameters while the effects of the motor training have not yet been fully addressed. The purpose of this study is to present a systematic review of all studies related to tDCS in conjunction with motor training (MT) to improve gait performance, functionality, mobility and balance in individuals with non-progressive central nervous system diseases.MethodsSeven databases were searched for articles from inception to October 2018. The search strategy followed Collaboration guidelines. The Physiotherapy Evidence Database (PEDro) Scale and Cochrane Collaboration's tool for assessing the risk of bias were applied to evaluate methodological quality.ResultsFour hundred and sixteen recorded were screened. Ten studies met the inclusion criteria. All studies were randomized controlled trials, two of them had a crossover design and other two were pilot studies. Three paper analyzed children and adolescents with cerebral palsy, seven papers analyzed adults and elderly post stroke. tDCS with MT lead to significant results.ConclusionsThis review found limited evidence for the use of tDCS with MT for in children with CP and adults post stroke, due to the small number of studies as well as their methodological heterogeneity. In the absence of more robust evidence, further studies with a consistent methodological design are needed to endorse the clinical application of tDCS with motor training.     

The value of elecromyography in the aetiological diagnosis of hypotonia in infants and toddlers

IntroductionDuring the first two years of life, hypotonia may be the only symptom of a central or peripheral nervous system disorder. We propose to assess the sensitivity of electroneuromyography (ENMG) in the aetiological diagnosis of hypotonia of neuromuscular origin in infants and toddlers.MethodThis is a retrospective, single-centre study with revision of the files of the 37 children aged between zero and 24 months who, between 1994 and 2006, underwent an ENMG in the etiological approach of their hypotonia and had a final diagnosis of neuromuscular disease.ResultsAll the 13 patients with spinal muscular atrophy or Charcot Marie-Tooth disease displayed neurogenic alterations on the electromyography (EMG). Among the 24 children ultimately diagnosed with myopathies, five only displayed myogenic alterations when tested before the age of two. Sixteen had normal EMG results and three showed neurogenic alterations.Discussion and conclusionIn infants presenting with hypotonia, ENMG is useful for the diagnosis of peripheral neuropathy. Normal ENMG is relatively common for confirmed muscle disorders in infants whereas myogenic alterations seem more unusual, so that muscle biopsy appears unquestionable. In a few cases, early onset myopathies may present with a neurogenic ENMG pattern. Such a result should not invalidate the clinically presumed diagnosis of myopathy and would indicate on the contrary the need for a muscle biopsy.     

Changes in Motor Unit Firing Rate in Synergist Muscles Cannot Explain the Maintenance of Force During Constant Force Painful Contractions

The firing rate of low threshold motor units is decreased in constant force contractions during experimental pain. However, as firing rate is a determinant of force, it is unclear how force is maintained. Increased synergist muscle activity may compensate. This was investigated by evaluation of motor unit firing rate in synergist ankle plantar flexor muscles (triceps surae). Single motor unit action potentials were recorded in medial gastrocnemius and soleus muscles with fine wire electrodes in 10 subjects. Gross muscle activity was estimated from surface electromyographic (EMG) recordings. Bolus injections of 5% hypertonic saline were injected into lateral gastrocnemius to induce pain (low intensity, 0.5 mL; high intensity, 1.5 mL). Subjects gently plantar-flexed the ankle to recruit 1 to 4 motor units and performed 3 20-second contractions to this target before, during, and after pain. Firing rate decreased ∼12% in synergist heads of triceps surae during pain and recovered after pain. Despite reduced firing rate, root-mean-square surface EMG amplitude did not change. The effect of nociceptor stimulation is not restricted to painful muscles but reduces motor unit firing in synergist muscles. Changes in synergist muscles cannot explain the maintenance of muscle force. Maintenance of surface EMG amplitude suggests recruitment of additional motor units.PerspectiveThis study showed that activity of synergist muscles can be affected by muscle pain. However, the changes in activity of synergist muscles may not compensate for changes in the painful muscle. This finding provides evidence of more widespread effects of pain on muscle control.     

Gene therapy for amyotrophic lateral sclerosis and other motor neuron diseases

There are several incurable diseases of motor neuron degeneration, including amyotrophic lateral sclerosis (ALS), primary lateral sclerosis, hereditary spastic hemiplegia, spinal muscular atrophy, and bulbospinal atrophy. Advances in gene transfer techniques coupled with new insights into molecular pathology have opened promising avenues for gene therapy aimed at halting disease progression. Nonviral preparations and recombinant adenoviruses, adeno-associated viruses, herpesviruses, and lentiviruses may ultimately transduce sufficient numbers of cerebral, brainstem, and spinal cord neurons for therapeutic applications. This could be accomplished by direct injection, transduction of lower motor neurons via retrograde transport after intramuscular injection, or cell-based therapies. Studies using transgenic mice expressing mutant superoxide dismutase 1 (SOD1), a model for one form of ALS, established that several proteins were neuroprotective, including calbindin, bcl-2, and growth factors. These same molecules promoted neuronal survival in other injury models, suggesting general applicability to all forms of ALS. Potentially correctable genetic lesions have also been identified for hereditary spastic hemiplegia, bulbospinal atrophy, and spinal muscular atrophy. Finally, it may be possible to repopulate lost corticospinal and lower motor neurons by transplanting stem cells or stimulating native progenitor populations. The challenge ahead is to translate these basic science breakthroughs into workable clinical practice.     

Vastus Lateralis Motor Unit Firing Rate Is Higher in Women With Patellofemoral Pain

Objective

To compare neural drive, determined from motor unit firing rate, in the vastus medialis and lateralis in women with and without patellofemoral pain.

Classification of Stroke Patients With Dysphagia Into Subgroups Based on Patterns of Submental Muscle Strength and Skill Impairment

ObjectivesTo identify and characterize subgroups of stroke patients with clinical signs of dysphagia, based on swallowing-related strength and skill impairments of the submental muscle group.DesignProspective observational study.SettingInpatient rehabilitation centers and community dwellings.ParticipantsIndividuals (N=114), including stroke patients with dysphagia (n=55) and 2 control groups including myopathic patients with dysphagia (n=19) and healthy volunteers (n=40) were included in this study.InterventionsNot applicable.Main Outcome MeasuresNovel clinical assessment of strength (force generation) and skill (spatial and temporal precision of muscle activation) of the submental muscle group during swallowing and nonswallowing behaviors, using surface electromyography and dynamometry.ResultsHierarchical cluster analysis revealed 4 clusters, which could be broadly characterized as cluster 1: intact strength and skill, cluster 2: poor strength and poor nonswallowing skill, cluster 3: poor strength, and cluster 4: poor strength and poor swallowing skill. Membership in cluster was significantly associated with medical diagnosis (P<.001). The majority of healthy and myopathic participants were assigned to clusters 1 and 3, respectively, whereas stroke patients were found in all 4 clusters. Skill outcome measures were more predictive of cluster assignment than strength measures.ConclusionsAlthough healthy and myopathic participants demonstrated predominantly homogeneous swallowing patterns of submental muscle function within their etiology, several subgroups were identified within stroke, possibly reflecting different subtypes of swallowing function. Future research should focus on the nature and rehabilitation needs of these subtypes. Assessment of skill in swallowing may be an important but overlooked aspect of rehabilitation.     

Effects of acupuncture treatment on motor function in patients with subacute hemorrhagic stroke: A randomized controlled study

ObjectiveThe aim of this study was to evaluate the additional effects of acupuncture treatment on motor function in patients with subacute hemorrhagic stroke.DesignOne hundred and thirty-four patients with subacute hemorrhagic stroke were randomized to receive acupuncture treatment plus conventional treatment (treatment group) or conventional treatment only (control group). Acupuncture treatments were given in 24 sessions over 4 weeks, with 3-month follow-up period. Blinded evaluation was based on Fugl-Meyer Assessment, Barthel Index with an intention-to-treat analysis. For those patients who were able to walk, a three-dimensional gait analysis system was employed to objectively record spatiotemporal and kinematic parameters.ResultsCompared with control group, the treatment group showed a significantly greater over-time improvement in total Fugl-Meyer, lower-limb Fugl-Meyer, but not in upper-limb Fugl-Meyer and Barthel Index. The spatiotemporal parameters of velocity, step length, cadence, step width all showed significant difference between the 2 groups. The velocity in treatment group decreased unexpectedly at day 14, then increased sharply and overcame control group at day 28. The treatment group also showed a significantly greater increase in peak circumduction, peak hip hiking, hip range of motion, knee range of motion and a tendency for the ankle range of motion.ConclusionsAcupuncture may promote the motor function recovery of hemorrhagic stroke patients in subacute phase mainly by enhancing the lower limb ability. It probably diminishes the compensation strategies earlier to correct the abnormal gait pattern. Although this adjustment may result in a compromise in the improvement of gait velocity temporarily, patients would benefit from it in a long run.     

成人型脊髓性肌萎缩症的骨骼肌病理与肌萎缩的关系

背景:脊髓性肌萎缩症是运动神经元疾病中病变仅影响下运动神经元的一组疾病。成人型少见,目前对其研究较少。目的:总结成人型脊髓性肌萎缩症骨骼肌病理学特征。设计:以诊断为依据的回顾性研究。地点和对象:收集1998-02/2002-02在解放军第八一医院南京医学院第二附属医院和南京军区总医院经肌肉活检确诊的、有完整临床资料的门诊和住院患者共46例。方法:结合临床特征及病理学改变进行分析。主要观察指标:病史、家族史、完整体格检查、相关血液及血生化、肌电图和肌肉活检。结果:临床表现为进行性对称性肢体近端肌萎缩,肌无力,实验室检查血肌酸磷酸肌酶12例中轻度升高,肌电图检查2例正常,3例呈轻度肌源性损害,余37例呈神经元性损害,肌活检主要为小群性肌萎缩,腺苷三磷酸酶染色见同型肌群化及肌纤维代偿性肥大。结论:肌活检对成人型脊髓性肌萎缩症具有诊断和鉴别诊断意义。适当、持久的康复锻炼可能对维持患者的运动功能有帮助。     

Androgen receptor function in motor neuron survival and degeneration

Polyglutamine repeat expansion in the androgen receptor is responsible for the motor neuron degeneration in X-linked spinal and bulbar muscular atrophy (SBMA; Kennedy's disease). This mutation, like the other polyglutamine repeat expansions, has proven to be toxic itself by a gain-of-function effect; however, a growing body of evidence indicates that loss of androgen receptor normal function simultaneously contributes to SBMA disease pathology, and, conversely, that normal androgen receptor signaling mediates important trophic effects upon motor neurons. This review considers the trophic requirements of motor neurons, focusing upon the role of known neurotrophic factors in motor neuron disease natural history, and the interactions of androgen receptor signaling pathways with motor neuron disease pathogenesis and progression. A thorough understanding of androgen receptor signaling in motor neurons should provide important inroads toward the development of effective treatments for a variety of devastating motor neuron diseases.     

New developments in exon skipping and splice modulation therapies for neuromuscular diseases

Introduction: Antisense oligonucleotide (AON) therapy is a form of treatment for genetic or infectious diseases using small, synthetic DNA-like molecules called AONs. Recent advances in the development of AONs that show improved stability and increased sequence specificity have led to clinical trials for several neuromuscular diseases. Impressive preclinical and clinical data are published regarding the usage of AONs in exon-skipping and splice modulation strategies to increase dystrophin production in Duchenne muscular dystrophy (DMD) and survival of motor neuron (SMN) production in spinal muscular atrophy (SMA).

Areas covered: In this review, we focus on the current progress and challenges of exon-skipping and splice modulation therapies. In addition, we discuss the recent failure of the Phase III clinical trials of exon 51 skipping (drisapersen) for DMD.

Expert opinion: The main approach of AON therapy in DMD and SMA is to rescue (‘knock up’ or increase) target proteins through exon skipping or exon inclusion; conversely, most conventional antisense drugs are designed to knock down (inhibit) the target. Encouraging preclinical data using this ‘knock up’ approach are also reported to rescue dysferlinopathies, including limb-girdle muscular dystrophy type 2B, Miyoshi myopathy, distal myopathy with anterior tibial onset and Fukuyama congenital muscular dystrophy.     

Assessing motor deficits in compressive neuropathy using quantitative electromyography

Background  

Studying the changes that occur in motor unit potential trains (MUPTs) may provide insight into the extent of motor unit loss and neural re-organization resulting from nerve compression injury. The purpose of this study was to determine the feasibility of using decomposition-based quantitative electromyography (DQEMG) to study the pathophysiological changes associated with compression neuropathy.