Muscle & Nerve最新文献

{"title":"Welcome From the Guest Editors and Meeting Co-Chairs.","authors":"Carolina Barnett-Tapia, Maartje G Huijbers","doi":"10.1002/mus.70363","DOIUrl":"https://doi.org/10.1002/mus.70363","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":"74 Suppl 1 ","pages":"S5-S6"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149025514","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":"NMES-Facilitated Mandibular Rehabilitation for Spasticity-Related Trismus in ALS.","authors":"Kelly Salmon, Hristelina Ilieva, Daniel Kelly","doi":"10.1002/mus.70332","DOIUrl":"10.1002/mus.70332","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"747-750"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13471755/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148369445","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":"Comparison of Consumer Smartwatch and Research-Grade Accelerometer-Derived Step Counts in Amyotrophic Lateral Sclerosis.","authors":"Gordon Jewett, Reed Ferber, Colin B Josephson, Lawrence Korngut, Joon Lee","doi":"10.1002/mus.70323","DOIUrl":"10.1002/mus.70323","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Objective, scalable measures of function are needed in amyotrophic lateral sclerosis (ALS). Research-grade accelerometers are promising but may be difficult to deploy for prolonged remote monitoring, whereas consumer smartwatches offer a practical alternative. We evaluated the feasibility of long-term smartwatch monitoring in ALS and compared smartwatch- and accelerometer-derived daily step counts.</p><p><strong>Methods: </strong>In this single-centre prospective observational study, participants wore a Fitbit Sense (smartwatch) on the wrist longitudinally and an ActiGraph GT9X Link (accelerometer) on the opposite wrist during clinic-aligned assessment periods. Device comparison was restricted to concurrent valid days (≥ 10 h wear) and included paired comparison of mean daily step counts, Pearson correlation, Bland-Altman analysis, and proportional bias assessment.</p><p><strong>Results: </strong>Forty participants were enrolled and 39 contributed data across 8093 smartwatch and 915 accelerometer days. Step count comparison included 503 concurrent valid days from 34 participants. Mean daily step counts were 3480 (SD 4365) for smartwatch and 4066 (SD 4797) for accelerometer (p < 0.0001), with strong correlation (r = 0.91, p < 0.0001). Mean bias was -586 steps for smartwatch, with 95% limits of agreement from -4193 to 3021. Among participants with ≥ 6 months of concurrent data, devices showed similar decline over time (interaction p = 0.76).</p><p><strong>Discussion: </strong>Long-term remote monitoring using a smartwatch was feasible in ALS. Smartwatch-derived step counts were strongly correlated with accelerometer-derived estimates but were systematically lower and showed wide day-level limits of agreement. Smartwatch-derived step counts may be useful for group-level analyses and longitudinal monitoring, though device-specific bias should be considered in interpretation.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"580-589"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13471975/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148284002","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 Anterior Belly of the Digastric Muscle as a Novel Target for Needle Electromyography in Amyotrophic Lateral Sclerosis.","authors":"Tatsuya Fukumoto, Yusuke Osaki, Kenta Hanada, Koji Fujita, Ryuji Kaji, Yuishin Izumi","doi":"10.1002/mus.70354","DOIUrl":"10.1002/mus.70354","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Needle electromyography (EMG) in the bulbar region aids in the diagnosis of amyotrophic lateral sclerosis (ALS) but remains technically challenging. The genioglossus muscle (GM) is commonly examined; however, pain and poor relaxation may limit its evaluation. The trapezius is an alternative target but is innervated by the spinal accessory nerve. We investigated the utility of the anterior belly of the digastric muscle (ABDM), innervated by the trigeminal nerve, for bulbar EMG evaluation.</p><p><strong>Methods: </strong>In this unblinded, prospective observational study, consecutive patients with suspected ALS underwent needle EMG of the ABDM, GM, masseter muscle (MM), and trapezius. Relaxation levels, EMG abnormalities, and pain (100-mm visual analog scale [VAS]) were assessed.</p><p><strong>Results: </strong>Twenty-seven clinically diagnosed ALS patients were analyzed. Relaxation was better in the ABDM than in the GM (p < 0.001) and comparable to that in the MM and trapezius. Fasciculation potentials were detected more often in the ABDM (52%) than in the GM (0%) and MM (22%; p = 0.039) and were comparable to the trapezius (59%). Chronic denervation occurred at similar frequencies across muscles (33%-59%). The combination of abnormal spontaneous activity and chronic denervation was most frequent in the ABDM (37%). Pain was lower with the ABDM (median VAS score of 21) than with the GM (28; p = 0.0052) and MM (32; p < 0.001) and was similar to the trapezius (23).</p><p><strong>Discussion: </strong>The ABDM may be a practical additional target for bulbar EMG in ALS, with favorable relaxation, lower pain, and potentially improved electrodiagnostic yield.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"685-691"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13471820/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148549901","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":"Cytokine Profiles in Myasthenia Gravis Subgroups and the Lack of Any Effect of Immunosuppression.","authors":"Merve Cebi, Hacer Durmus, Arman Çakar, Mehmet Güven Günver, Yesim Parman, Feza Deymeer, Güher Saruhan-Direskeneli","doi":"10.1002/mus.70278","DOIUrl":"10.1002/mus.70278","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Myasthenia gravis (MG) is a clinically and immunologically heterogeneous autoimmune disease and there is little known about the differential effects of cytokines in disease subgroups. This study aimed to compare serum cytokine profiles across distinct MG subgroups, including early-onset MG (EOMG), late-onset MG (LOMG), muscle-specific kinase antibody-positive MG (MuSK-MG), thymoma-associated MG (TAMG), and healthy controls (HC) in order to distinguish and determine relatively specific targets for the disease subgroups.</p><p><strong>Methods: </strong>Serum samples from 22 EOMG, 23 LOMG, 17 MuSK-MG, 25 TAMG patients, and 25 HC were analyzed for a panel of cytokines involved in T and B cell interactions using multiplex bead-based immunoassays and ELISA. Additionally, paired serum samples of the same patients before and after immunosuppressive therapy were evaluated in a subset of patients.</p><p><strong>Results: </strong>IL-17A, IL-4, IFN-γ, IL-21, and IL-9 were significantly elevated in EOMG and MuSK-MG groups, whereas IL-17F, IL-37, IL-2, TNF-α, and IL-13 were higher in EOMG only compared with HC. Increased levels of IL-6 in MuSK-MG and IL-37 in EOMG were found in these subtypes. In contrast, LOMG and TAMG patients showed limited cytokine alterations, with significant increases only in IL-21 in LOMG and IL-17A in TAMG. Immunosuppressive therapy did not significantly alter serum cytokine concentrations.</p><p><strong>Discussion: </strong>The findings reveal heterogeneous cytokine signatures among diverse MG subgroups, separating EOMG and MuSK-MG from TAMG and LOMG with some similarities and highlighting the impact of age and thymoma presence on immune responses. This assessment of disease subgroups has provided a broader understanding of the cytokines in MG.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"S142-S149"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13599451/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148361679","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":"Correction to Muscle & Nerve Articles.","authors":"","doi":"10.1002/mus.70377","DOIUrl":"https://doi.org/10.1002/mus.70377","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":"74 Suppl 1 ","pages":"S159"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149025492","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":"Optimizing Research Operations and Resource Utilization in ALS Care: Insights From the Tofersen Antisense Oligonucleotide Expanded Access Protocol.","authors":"Alison Wheeler, Kush Mehta, Danica Sanders, Cathy Chin, Hannah Zivalic, Judith Carey, Alexandra McCaffrey, Pravin Pant, Carl Lewenhaupt, Sarah Luppino, Gabriel Jacobs, Shea Golden, Ryan Gifford, Erica Scirocco, Mackenzie Keegan, Munaf Hatem, Taravat Yazdanian, Sanem P Uysal, Natalie Susco, Shauna Schwartzman, Karen Branch, Kathryn E Hall, Joanna Barnas, Jenny Lam, Jennifer Hagar, Carol Ann Hannan, Sabrina Paganoni, James D Berry, Mark Garret, Jennifer Scalia, Suma Babu","doi":"10.1002/mus.70310","DOIUrl":"10.1002/mus.70310","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Tofersen is a gene-targeted therapy for individuals with superoxide dismutase 1 (SOD1) (+) amyotrophic lateral sclerosis (ALS). Prior to U.S. Food and Drug Administration (FDA) approval, tofersen was made available through expanded access protocol. This study describes the clinical and operational experience of administering tofersen through expanded access protocols at a single academic medical center in the U.S.</p><p><strong>Methods: </strong>Individuals with symptomatic SOD1(+) ALS (≥ 18 years), who were ineligible for traditional ALS clinical trials, received tofersen via bedside lumbar punctures at Massachusetts General Hospital. Treatment was provided through single-patient and intermediate-sized expanded access protocols prior to FDA approval. Demographic and clinical characteristics, referral-to-treatment timelines, safety outcomes, and operational costs were collected.</p><p><strong>Results: </strong>Eleven individuals with SOD1(+) ALS received monthly intrathecal tofersen over a two-year period (July 2021 to July 2023). Most participants were female, and 81.8% had leg-onset ALS. The mean (SD) referral-to-first dose duration was 36 (22.4) days. A total of 120 doses were administered over a two-year period. Tofersen was safe and well tolerated, with no treatment-related serious adverse events. Operational costs totaled $336,620, supported by philanthropy and insurance. The company provided the drug for free.</p><p><strong>Discussion: </strong>This experience demonstrates the feasibility of implementing a resource-intensive expanded access protocol within an academic medical center using a mixed funding model to facilitate early access to emerging ALS therapies.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"692-697"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13472014/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148239740","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":"Successful Bortezomib-Lenalidomide-Dexamethasone Therapy in a 72-Year-Old Patient With Sporadic Late-Onset Nemaline Myopathy Associated With Monoclonal Gammopathy of Muscle Significance.","authors":"Hiroki Komatsu, Shogo Komaki, Meiko Maeda, Akatsuki Kubota, Mitsuhiro Yuasa, Mineo Kurokawa, Ryo Morishima, Satoko Uruha, Akinori Uruha, Ichizo Nishino, Masashi Hamada, Tatsushi Toda, Wataru Satake","doi":"10.1002/mus.70342","DOIUrl":"10.1002/mus.70342","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"756-758"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13471892/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148422791","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":"Cure in Myasthenia Gravis: An Unmet Need.","authors":"Shadi El-Wahsh, Fiona Chan, Stephen Reddel","doi":"10.1002/mus.70255","DOIUrl":"10.1002/mus.70255","url":null,"abstract":"<p><p>A cure for myasthenia gravis is necessary to achieve \"A World Without Myasthenia Gravis\". Current treatments reduce symptom burden and broadly suppress immune activity, but generally need to be administered chronically and are associated with significant risks, incomplete remission rates, infrequent treatment-free remission, and potential disability. We define a cure for MG as complete remission of MG, without the need for ongoing treatment, plus desirably: a high probability of success; a low risk of later relapse; the possibility of treatment repetition if MG does relapse; low and proportionate treatment toxicity; and cost-effectiveness. Thymectomy is the only currently available widely applied sometimes curative treatment for ACHR+ antibody MG. The MGTX trial post hoc analysis showed a 52% steroid free remission rate following thymectomy, which is approximately 1/3 of the whole population above the background remission rate. However, thymectomy is not universally applied even in younger ACHR+ patients. Recent regulatory approved and marketed therapies for MG are highly effective but are largely chronic continuous suppressive strategies at significant cost. Non-selective ablative chemotherapy and transplant or CAR-T approaches to B or plasma cells in development have toxicity risks and may still require maintenance therapies to prevent relapse. Treatments specific only to the pathogenic antibodies or clones using antigen-bait, anti-idiotype, or toleragenic approaches are yet to be demonstrated in humans with spontaneously acquired myasthenia gravis. A cure for myasthenia gravis remains an unmet need.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"S108-S112"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148352577","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":"Impact of Sexuality in Patients With Motor Neuron Disease.","authors":"R Yolanda Morgado Linares, Íñigo Rojas-Marcos, Marco Mesa, Eva Martínez, Macarena Cabrera-Serrano, Carmen Paradas","doi":"10.1002/mus.70348","DOIUrl":"10.1002/mus.70348","url":null,"abstract":"<p><strong>Introduction: </strong>Sexuality is a relevant yet understudied aspect in people living with motor neuron disease (MND). This study aimed to assess whether sexuality in people with MND is influenced by motor, cognitive, behavioral, or mood disturbances, and to explore whether being sexually active impacts caregiver burden.</p><p><strong>Methods: </strong>We conducted a cross-sectional observational study involving participants with MND and their primary caregivers from three ALS multidisciplinary units in Seville, Spain. Data were collected between October 2018 and July 2021. Functional, cognitive, behavioral, and mood assessments were conducted. Sexuality was evaluated through sex-specific questionnaires. Caregiver burden was assessed using the Zarit Burden Interview.</p><p><strong>Results: </strong>Among 66 participants who completed the sexuality assessment, 42.9% reported sexual activity in the previous month. Of these, 70% had no sexual dysfunction, while 26.7% had moderate and 3.3% severe dysfunction. No correlation was found between sexual activity and motor function. Sexual activity was less frequent in participants with apathy (p = 0.026), depression (p = 0.021), and frontotemporal dementia (p = 0.013). Among sexually active patients, those with cognitive impairment-particularly memory dysfunction-had higher rates of orgasm difficulties (p = 0.019). No other clinical parameters were related to sexuality. Sexuality of participants with MND did not influence caregiver burden.</p><p><strong>Discussion: </strong>Sexuality in MND is more associated with cognitive and mood factors than with motor disability. Addressing sexuality in clinical practice may contribute to improving quality of life and should be integrated into the multidisciplinary care of MND patients.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"669-677"},"PeriodicalIF":2.9,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13471760/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148471917","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}