Muscle & Nerve最新文献

{"title":"Paraneoplastic Anti-Contactin-1 Autoimmune Nodopathy.","authors":"Cathy Meng Fei Li, Adrian Budhram, Chai W Phua, Christen Shoesmith","doi":"10.1002/mus.28401","DOIUrl":"https://doi.org/10.1002/mus.28401","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":"71 6","pages":"1135-1137"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143982003","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":"Disease Modification in SOD1-ALS With Tofersen May Result in Serious CNS Inflammation.","authors":"Stephen N Scelsa, Daniel J L MacGowan","doi":"10.1002/mus.28413","DOIUrl":"https://doi.org/10.1002/mus.28413","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":"71 6","pages":"928-931"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144008559","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":"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":"1096-1103"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12064370/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neuromuscular Ultrasound Training: Bridging the Gap.","authors":"Nens van Alfen, Eman A Tawfik","doi":"10.1002/mus.28391","DOIUrl":"10.1002/mus.28391","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"922-924"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143586330","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":"Longitudinal Outcomes Among Patients With Duchenne Muscular Dystrophy: A Canadian Retrospective Population-Based Study.","authors":"Christina Qian, Alexa C Klimchak, Shelagh M Szabo, Katherine L Gooch, Roxana Dragan, Heather J Prior, Jean K Mah","doi":"10.1002/mus.28368","DOIUrl":"10.1002/mus.28368","url":null,"abstract":"<p><strong>Aims: </strong>There are few long-term studies evaluating clinical outcomes and mortality among individuals with Duchenne muscular dystrophy (DMD); particularly using longitudinal health administrative claims data, reflecting populations managed in typical clinical practice. This study aimed to characterize DMD outcomes via a population-based database.</p><p><strong>Methods: </strong>Patients with DMD, diagnosed between 01/1979 and 03/2020 at ≤ 10 years of age, were identified using the Manitoba Population Research Data Repository housed at the Manitoba Centre for Health Policy. De-identified longitudinal administrative data from 1998 to 2020 were used to retrospectively assess frequencies and age at first observation of key DMD outcomes including scoliosis, cardiovascular-related complications, severe respiratory-related morbidities, and mortality. Survival analyses using Kaplan-Meier curves were used to describe attrition and estimate probability of patients remaining observation-free by age.</p><p><strong>Results: </strong>This study included 198 patients with median (IQR) follow-up of 9.6 (6.6-15.5) years. Corticosteroid use was observed in 26%, with a mean (SD) percentage of days covered of 31% (39%) from initiation to end of follow-up. Scoliosis observations were captured in 18% (median[IQR] age 12 [11-15] years at first observation), severe respiratory-related morbidities in 20% (14[6.5-18] years), and cardiovascular-related complications in 32% of the cohort (12.5[2-20.5] years). Mortality was observed in 14% of the cohort. Kaplan-Meier curves estimated 15% mortality by age 20 years and 20% by 25 years.</p><p><strong>Discussion: </strong>In a population-based data set with decades of follow-up, these data provide longitudinal observations of the substantial burden of DMD, and insight into contemporary estimates of mortality and treatment patterns in Canada.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"955-962"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12060602/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143409259","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":"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":"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":"1104-1112"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","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":"Nerve Ultrasound Detects Nerve Atrophy in Patients With Ataxia-Telangiectasia: A Pilot Study.","authors":"Antonio E Camelo-Filho, Pedro L G S B Lima, Rodrigo F da Rosa, Tito B S Soares, André L S Pessoa, Paulo R Nóbrega, Pedro Braga-Neto","doi":"10.1002/mus.28396","DOIUrl":"10.1002/mus.28396","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Ataxia-telangiectasia (A-T) is a genetic multisystem neurodegenerative disorder characterized by cerebellar ataxia, oculocutaneous telangiectasia, extrapyramidal involvement, peripheral sensorimotor neuropathy, immunodeficiency, pulmonary disease, and an increased risk of malignancy that ultimately determines the shortened lifespan in many patients. A-T nerve ultrasonographic characteristics remain underexplored. This pilot study aimed to characterize the ultrasonographic morphology of peripheral nerves in patients with A-T.</p><p><strong>Methods: </strong>Ultrasound cross-sectional areas (CSAs) of the median, ulnar, sural, and tibial nerves were obtained from three A-T patients and were compared to reference values. Nerve conduction studies and electromyography were also performed. Given the small sample size and the exploratory nature of this study, formal statistical analyses were not performed, and descriptive statistics were presented for the data.</p><p><strong>Results: </strong>Nerve CSAs in A-T patients were smaller than in healthy controls at all measurement sites.</p><p><strong>Discussion: </strong>Nerve ultrasound revealed atrophy in the peripheral nerves of A-T patients. This reduction in nerve size may distinguish A-T and highlights the utility of nerve ultrasound as a non-invasive diagnostic tool for peripheral sensorimotor neuropathy. These findings may have important implications for early detection in clinical practice.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1091-1095"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143630650","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":"Sensory Nerve Action Potential Analysis in a Cohort of Patients With Spinal Muscular Atrophy Aged 12 Years and Older.","authors":"Leandra A A Ros, Boudewijn T H M Sleutjes, H Stephan Goedee, Fay-Lynn Asselman, Inge Cuppen, Ruben P A van Eijk, W Ludo van der Pol, Renske I Wadman","doi":"10.1002/mus.28384","DOIUrl":"10.1002/mus.28384","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Survival Motor Neuron 1 (SMN1)-related spinal muscular atrophy (SMA) is characterized by α-motor neuron degeneration, with sensory function assumed to be clinically preserved. However, recent studies in severely affected patients and animal models have challenged this view. Therefore, we assessed the maximum sensory nerve action potential (SNAP) amplitude of the median nerve in patients with SMA and examined its changes during treatment with SMN-splicing modifying therapies.</p><p><strong>Methods: </strong>We longitudinally assessed median nerve maximum SNAPs in 103 genetically confirmed patients with SMA (types 1c-4, aged ≥ 12 years) before and approximately 1 year after treatment with nusinersen or risdiplam. For comparison, we included 53 age- and sex-matched healthy controls, using identical settings. We also compared data with reference values from a previously published cohort.</p><p><strong>Results: </strong>Maximum SNAPs were abnormal in 6 patients with SMA (6%), which was comparable to controls (8%), even when corrected for age. In patients younger than 50 years, abnormal maximum SNAPs were more prevalent in patients with SMA types 1 and 2. Maximum SNAPs were higher in SMA compared with controls. Maximum SNAPs showed an age-related decline in most cohorts, but the decline was steeper in patients with SMA type 1c. There was no difference in SNAPs after 1 year of treatment.</p><p><strong>Discussion: </strong>Our findings suggest the preserved sensory integrity of the median nerve in the majority of patients with SMA (94%), even in longstanding disease. The resilience of sensory neurons of the median nerve, and whether this extends to other peripheral nerves, warrants further investigation.</p><p><strong>Trial registration: </strong>The study was approved by the local medical ethics committee (no. 20-143) and registered in the Dutch registry for clinical studies and trials (www.toetsingonline.nl-NL72562.041.20, March 26, 2020).</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1016-1024"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12060638/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542757","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":"Myostatin Knockout Mice Have Larger Muscle Fibers With Normal Function and Morphology.","authors":"Hans Degens, Ketan Patel, A Matsakas","doi":"10.1002/mus.28389","DOIUrl":"10.1002/mus.28389","url":null,"abstract":"<p><strong>Introduction: </strong>We assessed whether muscle fibers in myostatin knockout (MSTN-/-) mice are just larger or also exhibit morphological, metabolic, and functional differences from MSTN+/+ mice.</p><p><strong>Methods: </strong>We compared single fiber contractile properties and histological fiber properties in muscles from MSTN-/- and MSTN+/+ mice.</p><p><strong>Results: </strong>Even though in permeabilized muscle fibers from the extensor digitorum longus and soleus muscle maximal force was higher (p < 0.001) there were no significant differences in specific power (power per unit volume), specific tension (force per cross-sectional area), maximal shortening velocity, or curvature of the force-velocity relationship between MSTN-/- and MSTN+/+ mice. In histological sections of the soleus muscle, fibers were larger (p < 0.001), but the succinate dehydrogenase staining intensity and capillary density did not differ significantly between MSTN-/- and MSTN+/+ mice, which was explicable by the larger number of capillaries around a fiber (p < 0.001). A model showed no significant differences in soleus muscle oxygenation.</p><p><strong>Discussion: </strong>The larger force-generating capacity of fibers from MSTN-/- mice is explicable by the larger fiber cross-sectional area. The data indicate that muscle fibers from MSTN-/- mice are quantitatively, but not qualitatively different from muscle fibers from MSTN+/+ mice. Myostatin inhibition may help increase muscle mass in conditions accompanied by muscle weakness without a detrimental impact on muscle quality, but systemic side effects need to be considered.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1122-1131"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12060642/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143542751","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":"Sustained Minimal Manifestations With Extended Interval Dosing of Efgartigimod in Seronegative Myasthenia Gravis: Case Report.","authors":"Teppei Komatsu, Haruhiko Motegi, Yoshitaka Nakayama, Hiromasa Matsuno, Hidetaka Mitsumura, Yasuyuki Iguchi","doi":"10.1002/mus.28388","DOIUrl":"10.1002/mus.28388","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1132-1134"},"PeriodicalIF":2.8,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557455","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}