Muscle & NervePub Date : 2025-11-01Epub Date: 2025-09-10DOI: 10.1002/mus.70020
Jonathan M Puder, Albina Jablonka-Shariff, Matthew D Wood, Alison K Snyder-Warwick
{"title":"Brief Electrical Stimulation of the Distal and Proximal Sciatic Nerve During Transection and Repair in a Mouse Model.","authors":"Jonathan M Puder, Albina Jablonka-Shariff, Matthew D Wood, Alison K Snyder-Warwick","doi":"10.1002/mus.70020","DOIUrl":"10.1002/mus.70020","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Therapeutic electrical stimulation (ES) of repaired nerves has been demonstrated to improve muscle function. Previous studies applied ES to the proximal transected nerve end (P-ES) with benefits to the neuronal cell body. We investigated whether a single ES dose applied to the distal end (D-ES) or distal and proximal ends (DP-ES) prior to nerve repair provides benefits to neuromuscular junction (NMJ) and muscle recovery.</p><p><strong>Methods: </strong>S100-GFP mice were randomized to control (No ES), D-ES, P-ES, and DP-ES groups. After sciatic nerve transection, the ES groups received 10 min of ES prior to repair. The non-stimulated nerve end was insulated with a rubberized wrap. At 3 and 4 weeks post-repair, analyses using tetanic muscle force, grid walking, and NMJ immunohistochemistry were performed.</p><p><strong>Results: </strong>Muscle force demonstrated D-ES, P-ES, and DP-ES significantly improved muscle function at 4 weeks compared to No ES. Grid-walking tests showed significantly fewer foot faults in D-ES and DP-ES mice. Mice treated with ES showed higher NMJ innervation compared to No ES. In addition, the number of terminal Schwann cells (tSCs) and the percentage of NMJs with tSC sprouting beyond the NMJ area were significantly higher in ES groups, indicating improved innervation. Endplate morphology was similar among ES groups.</p><p><strong>Discussion: </strong>In addition to known benefits of P-ES, D-ES and P-ES demonstrated beneficial effects for reinnervation and muscle function post-nerve injury. While the mechanism is not fully characterized, ES applied to both D-ES and P-ES aspects of a transected nerve may act synergistically to improve muscle healing.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1178-1183"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145030178","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-11-01Epub Date: 2025-08-25DOI: 10.1002/mus.70009
Seward Rutkove, Gaurav Tiwari, Anjali K Nath
{"title":"Performing Compound Motor Action Potential Measurement in Zebrafish: A Description of Methodology and a Comparison Between Healthy and ALS-Affected Animals.","authors":"Seward Rutkove, Gaurav Tiwari, Anjali K Nath","doi":"10.1002/mus.70009","DOIUrl":"https://doi.org/10.1002/mus.70009","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The compound motor action potential (CMAP) is a very well-established output from standard motor conduction studies in patients. CMAP methods have also been developed for various animal models, including mice, rats, and dogs. Here, we describe a CMAP methodology for adult zebrafish.</p><p><strong>Methods: </strong>Using needle stimulating electrodes placed in proximity to the caudal spinal column and a fixed two-electrode surface array placed near the dorsal fin for recording, we obtained CMAPs in wildtype (WT) and symptomatic amyotrophic lateral sclerosis (ALS) SOD1<sup>G93A</sup> zebrafish, assessing repeatability and the potential for identifying differences between the groups.</p><p><strong>Results: </strong>In WT animals, CMAP amplitude exhibited robust performance with a test-retest intra-class coefficient of 0.97 (95% confidence interval 0.947-0.988; p < 0.0001, n = 30). SOD1<sup>G93A</sup> zebrafish exhibited a 36% lower supramaximal CMAP amplitude as compared to WT (mean ± standard deviation: 7.7 ± 1.7 mV versus 12.2 ± 1.8 mV, respectively, p < 0.0001) and an 11% longer latency (1.30 ± 0.15 ms versus 1.17 ± 0.11 ms, p = 0.002). A classifier, incorporating amplitude and latency together, provided perfect discrimination between the two cohorts.</p><p><strong>Discussion: </strong>CMAP recording is a reliable technique in zebrafish and can successfully differentiate healthy WT fish from ALS-affected animals. Since CMAP is a quantitative metric that is highly sensitive to motor neuron loss or dysfunction, it will allow the zebrafish to be more effectively harnessed for physiological and clinical therapeutic studies in ALS and other neuromuscular diseases for which adult zebrafish models are available.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":"72 5","pages":"1168-1177"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145301988","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-10-17DOI: 10.1002/mus.70045
Yuebing Li, David Bruckman, Jesse D Schold, Benjamin Claytor, Nicholas Silvestri, Michael K Hehir, Ikjae Lee
{"title":"Survival Outcomes of Medicare-Covered Elderly US Population With Myasthenia Gravis.","authors":"Yuebing Li, David Bruckman, Jesse D Schold, Benjamin Claytor, Nicholas Silvestri, Michael K Hehir, Ikjae Lee","doi":"10.1002/mus.70045","DOIUrl":"https://doi.org/10.1002/mus.70045","url":null,"abstract":"<p><strong>Introduction/aim: </strong>Survival outcomes have been inadequately studied among people with myasthenia gravis (MG) in the United States (US). We examined the impact of MG and comorbid conditions on longevity.</p><p><strong>Methods: </strong>We performed a longitudinal study using Medicare claims data (2006-2019). Incident MG cohort was identified based on the following criteria: age ≥ 65 years; ≥ 1 month of fee-for-service Parts A and B coverage; no health maintenance organization coverage; initial and subsequent MG claims within 2010-2011 separated by ≥ 28 days. A non-MG cohort of five times the number of the MG group was selected, matching for age, sex, region, and Medicare coverage duration. Overall and cause-specific mortality were compared between cohorts in the subsequent 8-10 Years using Kaplan-Meier plots and Cox proportional hazard models, adjusted for the Charlson Comorbidity Index (CCI).</p><p><strong>Results: </strong>Cohorts of 6024 incident MG and 30,083 control beneficiaries were Included. The mortality rate was higher in the MG cohort compared to controls (66.8 vs. 57.1 per 1000-person-year, p < 0.0001). After adjusting for time-varying CCI, no significant difference in survival was observed between two cohorts (adjusted hazard ratio 1.09 [0.87-1.36], p = 0.47). Sixteen percent of deaths in the MG cohort were attributed to MG. Compared to the non-MG cohort, mortality rates (per 1000-person-year) specific to infections were higher among the MG cohort (2.0 vs. 1.2) while malignancies and dementia-specific mortality rates were lower (10.3 vs. 12.5 and 4.7 vs. 7.2), all p < 0.01.</p><p><strong>Discussion: </strong>Long-term mortality is increased in elderly MG patients compared to non-MG counterparts, driven by their higher comorbidity burden.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":3.1,"publicationDate":"2025-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145308595","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-10-17DOI: 10.1002/mus.70048
{"title":"Correction to \"Characteristics of After-Discharges Following Compound Muscle Action Potential or F-Wave in Primary Peripheral Nerve Hyperexcitability Syndrome\".","authors":"","doi":"10.1002/mus.70048","DOIUrl":"https://doi.org/10.1002/mus.70048","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":3.1,"publicationDate":"2025-10-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145308570","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-10-09DOI: 10.1002/mus.70046
Antoine Pegat, Antoine Gavoille, Florent Cluse, Martin Moussy, Philippe Petiot, Jean-Philippe Camdessanché, Françoise Bouhour
{"title":"Sural/Radial Amplitude Ratio: A Useful Tool to Diagnose Non-Length-Dependent Neuropathy.","authors":"Antoine Pegat, Antoine Gavoille, Florent Cluse, Martin Moussy, Philippe Petiot, Jean-Philippe Camdessanché, Françoise Bouhour","doi":"10.1002/mus.70046","DOIUrl":"https://doi.org/10.1002/mus.70046","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Patients with non-length-dependent neuropathy (NLDN) exhibit reduced sensory nerve action potential (SNAP) amplitudes in both lower and upper limbs. This study aimed to determine a threshold for the sural/radial amplitude ratio (SRAR) suggestive of NLDN.</p><p><strong>Methods: </strong>This retrospective case-control study involved 60 patients with definite NLDN (sensory neuronopathy [SNN] or chronic inflammatory demyelinating polyradiculoneuropathy [CIDP]) and 30 patients with length-dependent neuropathy (LDN). The diagnostic performance of SRAR was evaluated using the area under the curve (AUC) of the modeled receiver operating characteristic (ROC) curve. The presence of a length-dependent electrodiagnostic (EDX) pattern, defined as a sural SNAP amplitude lower than the radial one, was evaluated in each group.</p><p><strong>Results: </strong>SRAR could be calculated in 90/164 (54.9%) of patients screened. Among patients with NLDN, the median SRAR was 0.74 (IQR 0.50-1.00) compared to 0.17 (IQR 0.12-0.23) in patients with LDN. The ROC curve analysis for NLDN versus LDN yielded an AUC of 0.94 (95% CI, 0.883-0.979). The SRAR threshold of 0.33 provided a sensitivity of 84.4% (95% CI, 77.8%-90.9%), specificity of 86.9% (95% CI, 79.7%-94%). The length-dependent EDX pattern was observed in 100% (30/30) of LDN patients and 63% (38/60) of NLDN patients. Among these 38 patients with NLDN, SRAR exceeded 0.33 in 78.9% (30/38).</p><p><strong>Discussion: </strong>SRAR appears to be useful in the electrophysiological evaluation of neuropathies. In addition to usual diagnostic criteria, an SRAR > 0.33 may strongly suggest NLDN such as SNN or CIDP.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":3.1,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145251955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-10-09DOI: 10.1002/mus.70044
Javier Rodriguez-Falces, Mathew Piasecki, Silvia Recalde-Villamayor, Cristina Mariscal, Armando Malanda, Javier Navallas
{"title":"Motor Unit Loss and Remodeling in Patients With Chronic Radiculopathy Using the EMG Filling Analysis.","authors":"Javier Rodriguez-Falces, Mathew Piasecki, Silvia Recalde-Villamayor, Cristina Mariscal, Armando Malanda, Javier Navallas","doi":"10.1002/mus.70044","DOIUrl":"https://doi.org/10.1002/mus.70044","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The way in which the EMG signal is progressively filled with motor unit potentials during increasing voluntary contraction provides valuable information about the motor units (MUs) in a muscle. Here, we demonstrate the application of the EMG filling method to detect MU loss and remodeling in patients with chronic radiculopathy.</p><p><strong>Methods: </strong>Concentric needle EMG signals were recorded from the tibialis anterior of 19 radiculopathy patients and 18 healthy controls during voluntary ramped contractions increasing from 0 to maximum. The EMG filling process was analyzed by measuring the EMG filling factor and EMG amplitude during the contraction.</p><p><strong>Results: </strong>The way in which the filling factor increased with increasing force differed (p < 0.001) between healthy controls (exponential filling curve) and radiculopathy patients (linear curve). The extent to which the EMG envelope amplitude increased throughout the ramp contraction was markedly greater (p < 0.001) in healthy controls (6.1-fold increase) compared to patients (0.5-fold increase). The filling factor at maximal effort was significantly higher in healthy controls than in patients (0.53 ± 0.04 vs. 0.45 ± 0.09, p < 0.001). The EMG filling analysis revealed clear signs of MU loss and remodeling in 85% of patients.</p><p><strong>Discussion: </strong>The EMG filling analysis is a fast and accessible approach to detect MU loss in radiculopathy patients. Moreover, it provides information about the MU remodeling process and the type of motor units that are predominantly lost in the neurogenic condition.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":3.1,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145251951","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-10-06DOI: 10.1002/mus.70043
Halil Can Alaydin, Halit Fidanci
{"title":"The Role of the Cutaneous Silent Period in the Electrophysiological Diagnosis of Guillain-Barré Syndrome.","authors":"Halil Can Alaydin, Halit Fidanci","doi":"10.1002/mus.70043","DOIUrl":"https://doi.org/10.1002/mus.70043","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Electrophysiological assessment of small fiber neuropathy in Guillain-Barré syndrome (GBS) remains underexplored in the current literature. This study evaluated the cutaneous silent period (CSP) to assess small fiber involvement in GBS and its utility for differentiating subtypes.</p><p><strong>Methods: </strong>We conducted a prospective study of 27 patients with GBS (15 with acute inflammatory demyelinating polyneuropathy [AIDP] and 12 with other subtypes), 20 with diabetic polyneuropathy, and 20 healthy controls. CSP was recorded from the abductor pollicis brevis (APB) and tibialis anterior (TA) muscles using surface electrodes and standardized stimulation protocols.</p><p><strong>Results: </strong>CSP latencies were significantly prolonged in AIDP patients compared to healthy controls: median APB onset and offset latencies were 117.9 and 161.9 ms versus 69.9 and 123.5 ms, respectively (both p < 0.001), while median TA onset and offset latencies were 144.3 and 176.4 ms versus 106.3 and 137.1 ms (both p < 0.001). AIDP patients also showed longer median APB onset (117.9 vs. 78.2 ms; p < 0.01) and offset (161.9 vs. 130.9 ms; p < 0.05) latencies than other GBS patients. Median CSP duration was shorter in AIDP patients compared to healthy controls (39.2 vs. 57.4 ms; p < 0.05).</p><p><strong>Discussion: </strong>CSP assessment may complement standard nerve conduction studies by identifying thinly myelinated fiber involvement that helps distinguish demyelinating GBS subtypes. While these findings may not change disease management, they enhance understanding of pathophysiology and may facilitate earlier diagnosis.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":3.1,"publicationDate":"2025-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145233064","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Predicting Amyotrophic Lateral Sclerosis Mortality With Machine Learning in Diverse Patient Databases.","authors":"Ling Guo, Ian Qian Xu, Sonakshi Nag, Jing Xu, Josiah Chai, Zachary Simmons, Savitha Ramasamy, Crystal Jing Jing Yeo","doi":"10.1002/mus.28487","DOIUrl":"10.1002/mus.28487","url":null,"abstract":"<p><strong>Introduction: </strong>Predicting mortality in Amyotrophic Lateral Sclerosis (ALS) guides personalized care and clinical trial optimization. Existing statistical and machine learning models often rely on baseline or diagnosis visit data, assume fixed predictor-survival relationships, lack validation in non-Western populations, and depend on features like genetic tests and imaging not routinely available. This study developed ALS mortality prediction models that address these limitations.</p><p><strong>Methods: </strong>We trained Royston-Parmar and eXtreme Gradient Boosting models on the PRO-ACT database for 6- and 12-month mortality predictions. Each visit was labeled positive (for death) if death occurred within 6 or 12 months, negative if survival was confirmed beyond that, and excluded if follow-up was insufficient, assuming patients were alive up to their last recorded visit. Models were validated on independent datasets from the North American Celecoxib trial and a Singapore ALS clinic population. Feature importance and the impact of reducing predictors on performance were evaluated.</p><p><strong>Results: </strong>Models predicted mortality from any clinical visit with area under the curve (AUC) of 0.768-0.819, rising to 0.865 for 12-month prediction using 3-month windows. Albumin was the top predictor, reflecting nutritional and inflammatory status. Other key predictors included ALS Functional Rating Scale-Revised slope, limb onset, absolute basophil count, forced vital capacity, bicarbonate, body mass index, and respiratory rate. Models maintained robust performance on the independent datasets and after reducing inputs to seven key predictors.</p><p><strong>Discussion: </strong>These visit-agnostic models, validated across diverse populations, identify key prognostic features and demonstrate the potential of predictive modeling to enhance ALS care and trial design.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":"72 4","pages":"653-661"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12435129/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145065115","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-10-01Epub Date: 2025-07-10DOI: 10.1002/mus.28466
Yingkai Li, Srikanth Muppidi, Shakthi Unnithan, Hussein R Al-Khalidi, Samantha Masterson, Wendi Huff, Vern C Juel, Jeffrey T Guptill, Shruti M Raja
{"title":"COVID-19 Vaccination and Infection in Myasthenia Gravis: A Longitudinal Patient Survey.","authors":"Yingkai Li, Srikanth Muppidi, Shakthi Unnithan, Hussein R Al-Khalidi, Samantha Masterson, Wendi Huff, Vern C Juel, Jeffrey T Guptill, Shruti M Raja","doi":"10.1002/mus.28466","DOIUrl":"10.1002/mus.28466","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Patients with myasthenia gravis (MG) often receive immunosuppressive treatments. While approved COVID-19 vaccines effectively prevent severe infections, the impact of vaccination among immunocompromised MG patients remains unclear. This study explored vaccination status, adverse events, and post-vaccination outcomes among MG patients.</p><p><strong>Methods: </strong>A REDCap survey was conducted between September 21, 2021 and November 18, 2021 through the Myasthenia Gravis Foundation of America to collect data on demographic and disease characteristics, COVID-19 infections, vaccination status, and precaution strategies. A follow-up survey was available from January 11, 2022 to March 8, 2022.</p><p><strong>Results: </strong>A total of 1205 participants [65.7% female, average age 59.6 ± 15.4 years] completed the survey. Most were White or Caucasian (87.9%), 63.6% held a bachelor's degree or higher, and 41.9% were retired. Acetylcholine receptor (AChR) antibody positivity was reported by 47%, MuSK-Ab positivity by 6.1%, and 19.6% were seronegative. Most (75%) were on immunosuppressive therapy, and the vaccination rate was 91.5%. Among COVID-19 vaccine recipients, 61.5% reported adverse effects, while 8.5% of participants remained unvaccinated due to concerns about side effects (65%) and potential worsening of MG symptoms (70.9%). Post-vaccination COVID-19 infection rates dropped from 9.7% to 2.4%. Hospitalization decreased from 2.3% to 0.3%, and MG exacerbation rates from 5.5% to 1.4%.</p><p><strong>Discussion: </strong>COVID-19 vaccination acceptance is high among MG patients. Reported side effects in MG patients were comparable to those in the general population, and vaccination was associated with a reduced COVID-19 infection rate. Addressing concerns about vaccine side effects and providing efficacy data specific to MG could aid unvaccinated individuals in decision-making.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"597-605"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144600985","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Motor Unit Number Index (MUNIX) in Control Children: Reference Values and Reliability.","authors":"Christophe Boulay, Emilien Delmont, Frédérique Audic, Cécile Halbert, Sébastien Pesenti, Brigitte Chabrol, Béatrice Desnous, Shahram Attarian","doi":"10.1002/mus.28470","DOIUrl":"10.1002/mus.28470","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The motor unit number index (MUNIX) is recognized as a reliable electrophysiological biomarker, and reference values are available for healthy adults but not for children. The aim of this study was to determine reference MUNIX values in healthy children.</p><p><strong>Methods: </strong>Sixty control children (28 females) were grouped as under 5 years (n<sub>1</sub> = 16), 5-10 years (n<sub>2</sub> = 15), 10-15 years (n<sub>3</sub> = 20), and 15-17 years (n<sub>4</sub> = 9). MUNIX measurements were performed by an experienced and an inexperienced investigator in the abductor digiti minimi (ADM) and abductor pollicis brevis (APB) on both sides when possible.</p><p><strong>Results: </strong>The APB MUNIX increased by 6.5 [95% confidence interval, 3.2-9.7] per year of age, 1.0 [0.5-1.5] per cm, and 1.7 [0.4-4.0] per kg (p < 0.001 in each case), but was not independently associated with either of these three variables in multivariable analysis. The APB compound muscle action potential (CMAP) increased by 0.42 [0.30-0.52] per year of age, 0.06 [0.04-0.08] per cm, and 0.11 [0.07-0.14] per kg (p < 0.001 in each case), and increased with age independently of height and weight at borderline significance (p = 0.065). Measurement reproducibility was high for the experienced investigator, but lower between the experienced and the inexperienced investigator.</p><p><strong>Discussion: </strong>The high intra-investigator reproducibility for the experienced investigator indicates that these parameters are useful electrophysiological biomarkers for disease progression and potential treatment response.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"625-631"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12435161/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144675317","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}