Muscle & Nerve最新文献

{"title":"Investigating the Mechanism of Conditioning Versus Postoperative Electrical Stimulation to Enhance Nerve Regeneration: One Therapy, Two Distinct Effects.","authors":"Paige B Hardy, Bonnie Y Wang, K Ming Chan, Christine A Webber, Jenna-Lynn B Senger","doi":"10.1002/mus.28385","DOIUrl":"10.1002/mus.28385","url":null,"abstract":"<p><p>Regeneration after peripheral nerve injury is often insufficient for functional recovery. Postoperative electrical stimulation (PES) following injury and repair significantly improves clinical outcomes; recently, conditioning electrical stimulation (CES), delivered before nerve injury, has been introduced as a candidate for clinical translation. PES accelerates the crossing of regenerating axons across the injury site, whereas CES accelerates the intrinsic rate of axonal regeneration; thus, it is likely that their mechanisms are distinct. The large body of literature investigating the mechanisms of electrical stimulation has not differentiated between CES and PES. In this review, we investigate the CES and PES paradigms within the existing literature, distinguish their mechanistic insights, and identify gaps in the literature. A systematic literature review was conducted, selecting articles identifying the pro-regenerative effects of electrical stimulation in the setting of peripheral nerve injury. As a mechanistic template, both paradigms implicate cation channels for the initiation of numerous signaling pathways that together upregulate regeneration-associated genes. CES and PES feature some overlap; activation of PI3K and MAPK signaling pathways, and upregulation of BDNF, GAP43, and GFAP are similar. Currently, the inflammatory environment in which PES is administered predominantly differentiates these mechanisms. However, gaps within the literature complicate the comparison between paradigms. Systematic review revealed the mechanisms for both CES and PES paradigms remain fragmented; though much of the literature assumes the involvement of particular signaling pathways, the evidence remains limited. Though it is likely there is overlap between mechanisms, further investigation is needed.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"15-33"},"PeriodicalIF":3.1,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12138495/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143605867","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":"Strapped for Strength: A Comparison Study of Dynamometry Techniques to Evaluate Knee Extensor Strength in Inclusion Body Myositis.","authors":"Katie Schütze, Ian Cooper, Brook Galna, Kelly Beer, Timothy J Fairchild, Madeline Schopp, Anna Brusch, Merrilee Needham","doi":"10.1002/mus.28406","DOIUrl":"10.1002/mus.28406","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Accurate measurement of knee-extensor strength in people with inclusion body myositis (IBM) is vital to track disease progression and provide a standardized outcome for clinical trials. Isokinetic dynamometers are the current gold standard tool for measuring knee-extensor strength. A less costly, more portable tool would have more widespread clinical and research applications. Current practice is to use handheld dynamometry, but there are concerns around the accuracy of this method as it relies on precise operator technique. This study investigates whether stabilization of the handheld dynamometer with a strap (SSHD) improves agreement with the isokinetic dynamometer (IKD) for measurement of knee-extensor strength in IBM participants compared to operator-stabilized handheld dynamometry (OSHD).</p><p><strong>Methods: </strong>Fifteen IBM participants had bilateral knee-extensor force measured using three methods of dynamometry on the same day: the isokinetic dynamometer, operator-stabilized handheld dynamometry, and strap-stabilized handheld dynamometry. A crossover design was used to account for fatigue bias.</p><p><strong>Results: </strong>Intraclass correlations (ICC) indicated poor absolute agreement of the IKD with OSHD (Strong leg = 0.240, Weak leg = 0.328), which was better for the SSHD method. Using the SSHD reduced the bias (i.e., there was less underestimation of force) between SSHD and the IKD compared to OSHD and the IKD (p < 0.05), indicating that strap-stabilized handheld dynamometry improved agreement and intraclass correlations with the IKD compared with operator-stabilized dynamometry.</p><p><strong>Discussion: </strong>Strap-stabilized handheld dynamometry of knee extensor strength is feasible in IBM patients and may correlate better with isokinetic dynamometry than operator-stabilized handheld dynamometry, but larger studies are needed to confirm this finding.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"42-48"},"PeriodicalIF":2.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12138486/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143772456","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":"The Impact of Brain-Derived Neurotrophic Factor rs6265 (Val66Met) Polymorphism on Therapeutic Electrical Stimulation for Peripheral Nerve Regeneration: A Preclinical Study of Therapy-Genotype Interactions.","authors":"Jordan B Walters, Maria J Quezada, Suning He, Ciara S Haynes, Kathy Steece-Collier, Timothy J Collier, Caryl E Sortwell, Colin K Franz","doi":"10.1002/mus.28409","DOIUrl":"10.1002/mus.28409","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Therapeutic electrical stimulation (TES) shows promise in enhancing nerve regeneration, but outcomes vary widely. This study investigates the impact of the rs6265 single nucleotide polymorphism (SNP) on TES efficacy in a preclinical rat model and human stem cell-derived motor neurons.</p><p><strong>Methods: </strong>Wild-type (WT) and rs6265 variant rats underwent sciatic nerve transection and received either TES or sham treatment. Muscle reinnervation was assessed through compound muscle action potentials and muscle fiber cross-sectional area. Isogenic human iPSC-derived motor neurons were used to study activity-dependent brain-derived neurotrophic factor (BDNF) secretion.</p><p><strong>Results: </strong>WT rats that received TES (WT Estim) showed improved muscle cross-sectional area, electrophysiological muscle reinnervation, muscle fiber size, and NMJ reinnervation compared to Sham [p = 0.0468, p < 0.0001, p = 0.0160, p < 0.0001, respectively]. In contrast, rs6265 allele carriers did not experience a benefit of TES (rs6265 Estim) when compared to the Sham treatment [p = 0.9754, p = 0.8606, p = 0.9815, p = 0.1047, respectively]. Additionally, rs6265 allele carriers exhibited impaired activity-dependent BDNF secretion in vitro.</p><p><strong>Discussion: </strong>The rs6265 polymorphism influences TES efficacy, highlighting the need for personalized approaches in peripheral nerve injury (PNI) treatment. These findings suggest that genetic screening could optimize therapeutic outcomes. Understanding genetic factors affecting TES response can enhance treatment strategies for PNI, potentially improving patient recovery and reducing outcome variability.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"149-157"},"PeriodicalIF":2.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12138492/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144064289","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":"Incomplete Presentations in Typical Chronic Inflammatory Demyelinating Polyneuropathy: A Single-Center, Retrospective Study.","authors":"Young Gi Min, Irad Ahmed, Christina Englezou, Yusuf A Rajabally","doi":"10.1002/mus.28419","DOIUrl":"10.1002/mus.28419","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Incomplete forms of typical chronic inflammatory demyelinating polyneuropathy (CIDP) have recently been described, but their frequency and clinical characteristics are uncertain. This study aimed to describe a cohort of patients with incomplete typical CIDP.</p><p><strong>Methods: </strong>We retrospectively analyzed 64 consecutive treatment-naïve patients with CIDP. Phenotypes were classified based on detailed motor examinations, and clinical, electrophysiological, and therapeutic characteristics were compared.</p><p><strong>Results: </strong>Nineteen (30%) subjects with typical CIDP presented with an incomplete phenotype; 12 (63.2%) exhibited a proximal arm-sparing pattern, 3 (15.8%) a distal arm-sparing pattern, 3 (15.8%) a pure paraparetic form, and 1 (5.2%) had a pure proximal form. In cases without full motor recovery, 11 (68.8%) maintained their original phenotype, while the rest transitioned to the complete (18.8%) or to another incomplete form (12.5%) due to involvement of previously unaffected segments. Subjects with incomplete typical CIDP had milder pre-treatment disability and weakness compared to those with the complete form, while other clinical and electrodiagnostic features were comparable. As opposed to the complete form, disability in incomplete typical CIDP at diagnosis showed no correlation with muscle strength.</p><p><strong>Discussion: </strong>Incomplete forms were observed in nearly one-third of subjects with typical CIDP. Incomplete typical CIDP represents a milder form of complete typical CIDP; however, its other disease characteristics, including treatment response, are similar, highlighting the importance of its proper prompt recognition as CIDP. Impairments beyond motor weakness, such as more diffuse proprioceptive loss, might play a role in the disability of patients with incomplete typical forms of CIDP.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"66-70"},"PeriodicalIF":2.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12138482/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144036141","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":"Physical Activity and Sedentary Behavior in Norwegian Boys With Duchenne Muscular Dystrophy: A Cross-Sectional Study.","authors":"Stian Hammer, Michel Toussaint, Haakon Kvidaland, Ola Drange Røksund, Bjørge Herman Hansen, Lars Peder Bovim, Jostein Steene-Johannessen, Karl Ove Hufthammer, Maria Vollsæter, Tiina Andersen","doi":"10.1002/mus.28418","DOIUrl":"10.1002/mus.28418","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The World Health Organization (WHO) recommends that all children, including those with disabilities, should be physically active for optimal health benefits. The primary aim of this study was to quantify and describe overall physical activity (PA) levels and sedentary behavior in Norwegian boys with Duchenne muscular dystrophy (DMD), in comparison with healthy controls.</p><p><strong>Methods: </strong>Boys aged 5-18 years with DMD were enrolled in this cross-sectional study. Sedentary time, light PA, and moderate-to-vigorous physical activity (MVPA) were measured with use of an accelerometer for seven consecutive days. Data on PA were obtained via self-report questionnaires. Comparisons were drawn between ambulatory and non-ambulatory boys with DMD, and between ambulatory boys with DMD and healthy controls, with data retrieved from a database.</p><p><strong>Results: </strong>Twenty-eight boys with DMD participated, with a mean age of 12.6 (standard deviation 3.6) years, of whom 24 had accelerometry data. Ambulatory boys had higher PA levels compared with non-ambulatory boys (p = 0.008), and 53% of ambulatory boys met the WHO's recommendation for daily MVPA, whereas none of their non-ambulatory counterparts did. Boys with DMD were less physically active compared with healthy peers. Sedentary time was higher over weekends compared with weekdays, and increased with age both in the DMD group and in healthy controls.</p><p><strong>Discussion: </strong>Further research is needed to improve participation in PA by exploring barriers and facilitators, with a particular focus on non-ambulatory boys with DMD.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"82-92"},"PeriodicalIF":2.8,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144002455","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":"Feasibility and Tolerability of Subcutaneous Immunoglobulin via Manual Push Pre-Filled Syringes for Inflammatory Neuropathies: A Retrospective Cohort Study.","authors":"Hannah Smith, Susan Cooper, Ryan Yann Shern Keh, David Gosal, Tim Lavin","doi":"10.1002/mus.28421","DOIUrl":"10.1002/mus.28421","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Subcutaneous immunoglobulin (SCIG) is available as vials or pre-filled syringes (PFS) and can be administered via pump or manual push to treat inflammatory neuropathies, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). There are limited data on PFS-SCIG manual push in this population, and this administration method may be perceived as challenging as large volumes of SCIG are required for immunomodulation. Here, we evaluated the feasibility of PFS-manual push SCIG in patients with inflammatory neuropathies.</p><p><strong>Methods: </strong>Patients with inflammatory neuropathies receiving immunoglobulin therapy in a single center were identified via the National Immunoglobulin Database. Case notes were retrospectively evaluated for patient characteristics and immunoglobulin use data.</p><p><strong>Results: </strong>Thirty-six patients with inflammatory neuropathies being treated with PFS-SCIG were identified (CIDP: n = 29; multifocal motor neuropathy: n = 2; other: n = 5). Overall, 27 patients initiated SCIG with PFS-manual push. The remaining patients initiated SCIG with PFS-pump administration but, during the study, most (n = 7) switched to PFS-manual push, while one switched from PFS-manual push to PFS-pump. Five patients experienced adverse events, mostly associated with infusion-site swelling that could be managed by reducing infusion rates. Patient perception of PFS-manual push was positive, with multiple patients commenting on its convenience.</p><p><strong>Discussion: </strong>PFS-manual push SCIG appeared to be feasible and generally well-tolerated in patients with inflammatory neuropathies. Further studies should confirm PFS-SCIG manual push as a viable option for patients with inflammatory neuropathies and explore whether PFS might enhance treatment satisfaction and quality of life.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"134-138"},"PeriodicalIF":3.1,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12138485/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144029055","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":"Muscle MRI Changes in Nebulin-Related Nemaline Myopathy.","authors":"Yajie Wang, Yun Yuan, Zhiying Xie, Yanjuan Wang, Yakun Wu, Meng Yu","doi":"10.1002/mus.28465","DOIUrl":"https://doi.org/10.1002/mus.28465","url":null,"abstract":"<p><strong>Introduction/aims: </strong>NEB mutations are the most frequent causes of autosomal recessive subtypes of nemaline myopathies (NMs). There are few studies on muscle magnetic resonance imaging (MRI) changes in NEB-related NMs. We aimed to characterize the diagnostic and prognostic value of muscle MRI patterns in NEB-related NMs.</p><p><strong>Methods: </strong>Twenty-one patients with confirmed diagnoses of NEB-related NMs were enrolled. The relationships between MRI changes in lower extremity muscles and clinical features were investigated.</p><p><strong>Results: </strong>Most patients (18/21) presented with lower limb muscle weakness, with a distal predominance. In the lower limbs, the mean fatty infiltration score was greatest in the soleus (2.44), followed by the gastrocnemius (2.00) and gluteus maximus (2.05). The severity of fatty infiltration in the thigh muscles was correlated with age at the time of MRI (r = 0.569, p = 0.009) and disease duration from symptom onset to MRI (r = 0.597, p = 0.005). The mean fatty infiltration scores of the gluteus maximus, vastus medialis, sartorius, and adductor maximus were moderately correlated with disease duration (r-values = 0.467-0.597).</p><p><strong>Discussion: </strong>Our study revealed that the degree of lower limb muscle fatty infiltration in NEB-related NMs was correlated with the disease course, with the gluteus maximus of the thigh and soleus of the calf being the most severely affected. Edema of the lower limb muscles was also more common and noticeable in the calf than in the thigh. These muscle MRI changes may be helpful in the diagnosis and follow-up of this disease.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144529002","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":"Elevated Cancer Prevalence Identified at Specific Anatomical Sites Among People With Myotonic Dystrophy Using a Population-Based Sample.","authors":"Wanfang Zhang, Vinay Bhandaru, Nicholas E Johnson, Amy Moore, Abigail Lyons, Reba Berry, Christina Westfield, Daria McMahon, Aida Soim, Bo Cai, Suzanne McDermott","doi":"10.1002/mus.28464","DOIUrl":"https://doi.org/10.1002/mus.28464","url":null,"abstract":"<p><strong>Introduction/aim: </strong>To optimize patient care and cancer screening in myotonic dystrophy (DM), it is crucial to clarify cancer risks by DM type to guide targeted screening and prevention efforts. This study describes the prevalence of cancer among individuals diagnosed with DM from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet), and compares it to cancer prevalence in the US Surveillance, Epidemiology, and End Result (SEER) data.</p><p><strong>Methods: </strong>This retrospective cohort study used 2008-2019 medical records data on 1229 individuals diagnosed with DM (DM1: 81.4%, DM2: 9.4%, DM-not otherwise specified [NOS]: 9.2%) from MD STARnet. Age- and sex-specific cancer prevalence rates from SEER were applied to the MD STARnet data to calculate 12-year limited duration (LD) and complete standardized prevalence ratios (SPR) for total cancer and by primary cancer site.</p><p><strong>Results: </strong>Over a 12-year span, individuals with DM1 had a 4.01-fold higher LD prevalence of thyroid cancer and a 17.97-fold higher LD prevalence of nonmelanoma skin cancer compared to the SEER prevalence, respectively. Complete prevalence assessments identified that individuals with DM1 had a 4.18-fold higher prevalence of thyroid cancer, a 2.36-fold higher prevalence of melanoma, and a 4.68-fold higher prevalence of ovarian cancer. For DM2, the study observed a 34.02-fold higher LD prevalence for nonmelanoma skin cancer. Additionally, a significantly elevated complete prevalence of breast cancer (2.65-fold) was noted for DM2 compared to SEER data.</p><p><strong>Discussion: </strong>Future research with larger cohorts is needed to define cancer risks by DM subtype and investigate underlying biological mechanisms.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144529000","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":"Systemic Neisseria sicca Infection Associated With Ravulizumab Therapy in Myasthenia Gravis.","authors":"McKenzye M DeHart McCoyle, Iryna M Muzyka, Suraj Muley, Benn Smith, Alec Johnson, Sandhya R Nagarakanti","doi":"10.1002/mus.28454","DOIUrl":"https://doi.org/10.1002/mus.28454","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144285450","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":"Less Social Contact During the Pandemic Could Explain the Lower Prevalence of GBS During This Time.","authors":"Josef Finsterer","doi":"10.1002/mus.28457","DOIUrl":"https://doi.org/10.1002/mus.28457","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-06-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144266801","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}