Muscle & Nerve最新文献

{"title":"Physical Activity Correlates With Skeletal Muscle MRI Findings in Individuals With Duchenne Muscular Dystrophy.","authors":"Jaclyn Tamaroff, Nicholas Joy, Bruce Damon, Larry W Markham, Thomas Donnelly, Karry Su, Maciej S Buchowski, Kimberly Crum, James C Slaughter, Meng Xu, W Bryan Burnette, Jonathan Soslow","doi":"10.1002/mus.28323","DOIUrl":"10.1002/mus.28323","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Skeletal muscle magnetic resonance imaging (MRI) is a validated noninvasive tool to assess Duchenne muscular dystrophy (DMD) progression. There is interest in finding DMD biomarkers that decrease the burden of clinical trial participation, such as wearable devices. Our aim was to evaluate the relationship between activity, via accelerometry, and skeletal muscle MRI, particularly T₂ mapping.</p><p><strong>Methods: </strong>DMD children and young adults completed skeletal muscle MRI and were asked to wear an accelerometer on the dominant wrist for 7 days. MRI data included fat-suppressed transverse relaxation time (T₂) mapping of the calves and longitudinal relaxation time (T₁) mapping. Activity was assessed as vector magnitudes (VMs) and fraction of time (FOT) in activity groups (sedentary 1 or 2, low 1 or 2, moderate-to-vigorous physical activity (MVPA)).</p><p><strong>Results: </strong>Participants (n = 22; median age 11.4 years, 41% ambulatory) wore the accelerometer for a median of 7 days. Longer T₂ in multiple lower extremity muscles was negatively correlated with VMs per minute (tibialis posterior Spearman's rho = -0.68, p < 0.001), even when accounting for age, ambulatory status, or glucocorticoid use. Longer T₂ of the tibialis posterior was positively correlated with FOT in sedentary 1 (rho = 0.49, p = 0.02) and negatively correlated with FOT in higher activity levels (low 1 (rho = -0.58, p = 0.004), low 2 (rho = -0.67, p = 0.002), MVPA (rho = -0.7, p < 0.001)).</p><p><strong>Discussion: </strong>In individuals with DMD, longer T₂ on skeletal muscle MRI of the calves moderately correlated with lower activity levels indicating the potential use of home accelerometry as a future clinical trial biomarker of skeletal muscle health and progression in DMD.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"353-359"},"PeriodicalIF":2.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11799400/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142885879","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}

{"title":"Serious Neurologic Adverse Events in Tofersen Clinical Trials for Amyotrophic Lateral Sclerosis.","authors":"Alexandra Lovett, Sowmya Chary, Suma Babu, Gaëlle Bruneteau, Jonathan D Glass, Merete Karlsborg, Shafeeq Ladha, Keith Mayl, Christopher McDermott, Robert C Bucelli, Adriano Chiò, Toby A Ferguson, Thos Cochrane, Stephanie Fradette, Karen Smirnakis, Jennifer Inra, Sohail Malek, Laura Fanning","doi":"10.1002/mus.28372","DOIUrl":"https://doi.org/10.1002/mus.28372","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Tofersen is approved for the treatment of amyotrophic lateral sclerosis (ALS) due to superoxide dismutase 1 mutations (SOD1-ALS). Here we report serious neurologic adverse events (AEs) that occurred in the tofersen clinical trials in people with SOD1-ALS.</p><p><strong>Methods: </strong>Serious neurologic AEs of myelitis, radiculitis, aseptic meningitis, and papilledema reported in the tofersen clinical trials are described. Serious AEs were defined according to International Conference for Harmonization guidelines, and neurologic AEs in clinical trials were diagnosed by investigators based on symptoms, clinical examination findings, and diagnostic workup.</p><p><strong>Results: </strong>Ten participants (approximately 7% of tofersen 100-mg-treated trial participants) experienced a total of 12 serious neurologic AEs-4 of myelitis, 2 of radiculitis, 2 of aseptic meningitis, and 4 of intracranial hypertension (ICH) and/or papilledema. All events but one resolved either spontaneously, with dosing interruption/modification, or with concomitant therapies. One event was ongoing but improved as of December 2022. While 3 events led to tofersen treatment discontinuation, all other participants were able to remain on treatment. No event was life-threatening or fatal.</p><p><strong>Discussion: </strong>Some antisense oligonucleotides (ASOs) have been described as having pro-inflammatory properties. Aseptic meningitis has been reported with nusinersen; however, myelitis, radiculitis, increased intracranial pressure, and papilledema have not been reported with ASO treatment. These neurologic AEs should be considered when assessing the overall benefit/risk of tofersen treatment for SOD1-ALS. Safety data from the open-label extension and expanded access program will continue to characterize these events and further inform the safety profile of tofersen in SOD1-ALS.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143523532","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":"Frequent Tetanic Exercise Through Electrical Muscle Stimulation May Reduce Immobilization-Induced Muscle Fibrosis by Suppressing Myonuclear Apoptosis.","authors":"Yuichiro Honda, Moeka Yoshimura, Ayumi Takahashi, Seima Okita, Jumpei Miyake, Yudai Ishiki, Chiaki Seguchi, Junya Sakamoto, Minoru Okita","doi":"10.1002/mus.28381","DOIUrl":"https://doi.org/10.1002/mus.28381","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Immobilization-induced fibrosis is the primary pathogenesis of muscle contracture, and its trigger is myonuclear apoptosis. Tetanic exercise through electrical muscle stimulation may be able to mitigate myonuclear apoptosis; this could be an intervention strategy for Immobilization-induced fibrosis. In the present study, this was tested using rat skeletal muscles.</p><p><strong>Methods: </strong>Rats were divided into the control, immobilization, low-contraction frequency (LCF), and high-contraction frequency (HCF) groups. The soleus muscles were used as specimens.</p><p><strong>Results: </strong>The number of TUNEL-positive myonuclei was 0.36 ± 0.11, 4.66 ± 0.90, 4.25 ± 0.99, and 1.90 ± 0.46 in the control, immobilization, LCF, and HCF groups, respectively. The HCF group was lower than the immobilization and LCF groups (all p < 0.001). The number of myonuclei and cross-sectional area (CSA) in the HCF group was higher than in the immobilization and LCF groups (all p < 0.001). The number of macrophages, mRNA expression of IL-1β, TGF-β1, and α-SMA, and hydroxyproline contents in the HCF group was lower than in the immobilization and LCF groups (all p < 0.001). There were moderate to strong negative correlations between the number of TUNEL-positive myonuclei and the number of myonuclei and between the CSA and the number of macrophages. Moderate to strong positive correlations were found between the number of myonuclei and the CSA, the number of macrophages and IL-1β, IL-1β and TGF-β1, TGF-β1 and α-SMA, and α-SMA and hydroxyproline contents.</p><p><strong>Discussion: </strong>Frequent tetanic exercise might mitigate macrophage accumulation caused by myonuclear apoptosis and suppress immobilization-induced muscle fibrosis due to fibrosis-associated molecule overexpression.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516260","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":"Characteristics of Patients With Spinal Muscular Atrophy Who Have Switched Treatments: A Multi-Center Experience in the United Kingdom.","authors":"Emer O'Reilly, Georgia Stimpson, Annemarie Rohwer, Evelin Milev, Amy Wolfe, Giovanni Baranello, Francesco Muntoni, Mariacristina Scoto","doi":"10.1002/mus.28383","DOIUrl":"https://doi.org/10.1002/mus.28383","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The evolving landscape of spinal muscular atrophy (SMA) treatment in the United Kingdom allows patients to switch, with health authority approval, from one treatment to another. This retrospective analysis explores the characteristics of pediatric patients across the United Kingdom who switched between available therapies that included nusinersen, risdiplam, and onasemnogene abeparvovec (OA).</p><p><strong>Methods: </strong>Demographic data were collected, along with indicators of disease severity and motor function scores for all pediatric patients registered with the SMA REACH UK database who received nusinersen or risdiplam as initial treatments.</p><p><strong>Results: </strong>Data were available for 375 patients across 19 sites. In total, 289 patients received nusinersen (of whom 21% switched to risdiplam and 16% to OA), and 86 patients received risdiplam (of whom 5% switched to OA). The top reported reasons for treatment changes were as follows: nusinersen to risdiplam: related to spine complexity (28/52), other difficulties related to administration (23/52); nusinersen to OA: parent preference (19/36), bridging to OA (14/36); risdiplam to OA: parent preference (2/2). After switching, no patients requested to go back to the initial treatment. No patients demonstrated deterioration beyond the expected disease trajectory prior to switching.</p><p><strong>Discussion: </strong>The availability of treatments in the United Kingdom has fundamentally influenced treatment choices, but deterioration in a treatment was not identified as a factor in switching treatments. Further work will be needed to investigate the long-term trajectory of these patients.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516258","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":"Hydrodissection With or Without Corticosteroid Versus Corticosteroid-Only Injection for Carpal Tunnel Syndrome: Double-Blind Randomized Controlled Trial.","authors":"Sahar Ghorbanpour, Mina Abdi, Negin Naeemi, Sarvenaz Rahimibarghani, Maryam Hosseini, Seyede Zahra Emami Razavi, Mohaddeseh Azadvari","doi":"10.1002/mus.28382","DOIUrl":"https://doi.org/10.1002/mus.28382","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Hydrodissection is an emerging technique in the management of carpal tunnel syndrome. We aimed to compare the efficacy of hydrodissection with or without corticosteroid versus intra-carpal corticosteroid injection for treating carpal tunnel syndrome.</p><p><strong>Methods: </strong>In total, 66 wrists with mild to moderate carpal tunnel syndrome were allocated to three study arms. Under ultrasound guidance, patients received a single injection consisting of 1 mL (40 mg) triamcinolone acetonide, 1 mL lidocaine, and 3 mL of saline, or 1 mL lidocaine, and 4 mL saline. Another group received 1 mL intra-carpal triamcinolone acetonide (40 mg) without ultrasound guidance. All participants were followed for 3 months post-injection. The primary outcome was the median nerve cross-sectional area. Secondary outcomes were symptom severity, functional status, electrodiagnostic test results, hang grip, and pain.</p><p><strong>Results: </strong>Analyses showed a significant reduction in symptom severity, improvement in function, decrease in median cross-sectional area, and improvement in electrodiagnostic evaluations for all the interventions 3 months after the injections (all p < 0.05). However, there was no significant improvement in muscle strength with any of the interventions. Sensory distal latency decreased (p ≤ 0.004), and sensory nerve conduction velocity increased in all groups (p ≤ 0.001). For motor nerve evaluations, distal latency decreased significantly with the three interventions (p ≤ 0.003), while nerve conduction velocity in the forearm segment increased in the steroid-only group (p < 0.011). Group comparisons did not reveal any significant differences.</p><p><strong>Discussion: </strong>All interventions were effective in improving symptom severity, function, and electrodiagnostic and ultrasound measures, with no significant differences observed between the treatments.</p><p><strong>Trial registration: </strong>The trial was registered prospectively at the Iranian Registry of Clinical Trials website http://www.irct.ir/, a WHO Primary Register set up with the registration number IRCT20210613051566N1.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143502486","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":"Broadening Our Scope and Engaging Our Readers.","authors":"Zachary Simmons","doi":"10.1002/mus.28378","DOIUrl":"https://doi.org/10.1002/mus.28378","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143483643","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":"Efficient and Rapid Histomorphometry of Regenerating Peripheral Nerve.","authors":"Jenny Yau, Iván Coto Hernández, Steven Minderler, Keisha Barrera, Nate Jowett","doi":"10.1002/mus.28380","DOIUrl":"https://doi.org/10.1002/mus.28380","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Peripheral nerve surgery entails repairing or transferring nerves to restore sensory or motor functions. Functional outcomes correlate with axon counts within operated nerves, but there exists no reliable means for intraoperative assessment of injured nerves. Conventional histologic assessment of regenerating peripheral nerve is labor-intensive, requiring multiday processing for tissue immunohistochemistry. Here, we describe a high-throughput protocol for histomorphometry of regenerating peripheral nerves.</p><p><strong>Methods: </strong>Healthy and regenerating murine and human peripheral nerve sections were stained with commercial fluorescent membrane-specific dyes, one of which (MemBrite) was discovered to internalize within axons. Samples were imaged using confocal microscopy, and regenerating axons were quantified using commercial segmentation software. Quantification results using the novel staining protocol were compared against conventional neurofilament immunohistochemistry. The protocol was employed to guide intraoperative decision-making in a case of free functional muscle transfer for treating a patient with facial paralysis.</p><p><strong>Results: </strong>Axon quantification within healthy and regenerating nerve sections in human and murine samples via rapid staining yielded similar results when compared to antineurofilament labeling. The relative difference in axon counts for the healthy murine facial nerve for antineurofilament and MemBrite 594 staining was < 1%. For the regenerating human nerve, the relative difference was 8.7%.</p><p><strong>Discussion: </strong>A rapid quantification protocol for regenerating axons has been described, which may inform intraoperative decision-making and advance knowledge in peripheral nerve surgery. Minimizing tissue processing time during surgery would allow surgeons to receive immediate feedback regarding axon counts in the donor nerve for cross-facial nerve surgeries, ultimately leading to improved clinical outcomes for their patients.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143492990","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":"Multipoint Stimulation Motor Unit Number Estimation of the Extensor Indicis and Anconeus After Spinal Cord Injury.","authors":"Adenike A Adewuyi, Mahdis Hashemi, Shreyaa Khanna, Michael J Berger, Colin K Franz","doi":"10.1002/mus.28373","DOIUrl":"https://doi.org/10.1002/mus.28373","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Cervical spinal cord injury (SCI) significantly impairs upper limb function, affecting patients' quality of life. Nerve transfer surgery can restore arm and hand function, but its success depends on the health of infralesional lower motor neurons (LMNs). LMN abnormalities are prevalent in muscles targeted for nerve transfer, particularly those innervated by the posterior interosseous nerve (PIN) and radial nerve, essential for wrist extension and hand opening. This study evaluates the health of infralesional LMNs in cervical SCI using multipoint stimulation motor unit number estimation (MPS-MUNE). We assessed motor unit counts in the C7-innervated anconeus and the predominantly C8-innervated extensor indicis (EI) to determine their viability as targets for nerve transfer surgery.</p><p><strong>Methods: </strong>We conducted a prospective, two-center cohort study using MPS-MUNE to evaluate 15 individuals with cervical SCI (26 limbs) and 17 healthy controls.</p><p><strong>Results: </strong>Compound muscle action potential (CMAP) and MUNE values were significantly lower in SCI patients compared to controls (EI CMAP: 2.0 mV ± 1.57, EI MUNE: 33 ± 30.5; anconeus CMAP: 2.7 mV ± 1.9, anconeus MUNE: 39 ± 50.6 versus controls: EI CMAP: 6.6 mV ± 1.0, EI MUNE: 137 ± 33.9; anconeus CMAP: 6.6 mV ± 1.3, anconeus MUNE: 146 ± 42.3). Test-retest reliability for EI and anconeus were 0.84 (CI: 0.45-0.95) and 0.78 (CI: 0.36-0.93), respectively.</p><p><strong>Discussion: </strong>Significant LMN loss was observed after cervical SCI. MPS-MUNE shows potential for evaluating LMN health, highlighting its importance for assessing nerve transfer targets and planning interventions.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143483647","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":"Exercise as Medicine in Neuromuscular Disorders: Changing Attitudes, Ongoing Challenges, and Future Research Directions.","authors":"Matti D Allen, W David Arnold","doi":"10.1002/mus.28369","DOIUrl":"https://doi.org/10.1002/mus.28369","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143458694","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":"Update on Exercise in Persons With Muscle Disease.","authors":"Mansoureh Mamarabadi, Virginia Kudritzki, Yuebing Li, Ileana M Howard","doi":"10.1002/mus.28356","DOIUrl":"https://doi.org/10.1002/mus.28356","url":null,"abstract":"<p><p>Myopathies are heterogeneous in their etiology, muscle group involvement, clinical manifestation, and progression. Deficits in myopathy may include muscle weakness, atrophy, stiffness, myalgia, and extra-muscular manifestations. Consequently, these deficits could lead to impaired musculoskeletal function, inadequate engagement in daily activities and reduced participation in social activities. Exercise has been viewed as a potentially efficacious intervention to halt the loss of muscle function and to improve secondary symptoms that result from muscle loss, such as pain and fatigue. The purpose of this review is to discuss research findings within the last 10 years that examine effects of exercise interventions in many types of myopathies in humans. In general, most studies were small scale, and they varied with respect to exercise type, intensity, and outcome measures. Despite the different pathologies, various exercise subtypes of aerobic/endurance or strength/resistance training are generally beneficial and may improve muscle strength and functional outcomes. Exercise therapies are generally safe and well tolerated. Exercise prescription should be part of routine neuromuscular care for patients with myopathy, and ideally with input from a multidisciplinary team, with a focus on providing individualized exercise regimens. Further work is needed to define the optimal intensity and type of exercise to result in the best functional outcomes for persons with myopathy, as well as the effects of combining exercise and novel disease modifying therapies.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143458695","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}