Muscle & Nerve最新文献_第2页

Ultrasound-Guided Nerve Hydrodissection for Peripheral Entrapment Neuropathies. 超声引导下神经水解剖治疗周围压迫性神经病。

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-08-06 DOI: 10.1002/mus.28471

Berdale Colorado, Darien McNeill, John Norbury

{"title":"Ultrasound-Guided Nerve Hydrodissection for Peripheral Entrapment Neuropathies.","authors":"Berdale Colorado, Darien McNeill, John Norbury","doi":"10.1002/mus.28471","DOIUrl":"10.1002/mus.28471","url":null,"abstract":"Ultrasound-guided nerve hydrodissection is a technique whereby fluid is used to separate a nerve from surrounding tissue as a means to alleviate symptoms of peripheral entrapment neuropathies. Pre-procedure scans are necessary to plan a safe procedure. Typically, the operator will target the site of nerve entrapment, which is just distal to the point of maximum cross-sectional area enlargement. Possible mechanisms for improvement in symptoms include improvement in the function of the nervi nervorum and vasa nervorum. These procedures offer an option after conservative measures (such as splinting and activity modification) have failed but before surgical intervention is considered. They also can play a role in post-surgical entrapment (such as after a failed carpal tunnel release) when scar tissue contributes to the mononeuropathy. Anesthetic, normal saline, dextrose 5% in water (D5W), hyaluronic acid, platelet-rich plasma (PRP), and corticosteroid have all been reported as solutions used to hydrodissect nerves, and D5W may be the preferred injectate for injectate preparations not utilizing corticosteroid. This expert clinical perspective reviews the choice of injectate, needle tracking technique, and the state of the science with regard to hydrodissection in carpal tunnel syndrome. Ulnar neuropathy at the elbow, radial tunnel syndrome, saphenous neuropathy, sciatic neuropathy, and fibular neuropathy are other conditions that may be amenable to hydrodissection in select cases. Further research is needed to define the ideal volume of injectate for these procedures and to more accurately identify the patient populations who will benefit most from these procedures.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1052-1059"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144789606","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}

引用次数: 0

Cardiac Troponin T in Tofersen-Treated SOD1 ALS Patients: Beginning to Resolve the Catch-22 of Identifying Treatment Responsive Biomarkers in ALS Drug Development. tofersen治疗SOD1 ALS患者的心肌肌钙蛋白T：开始解决在ALS药物开发中识别治疗反应性生物标志物的第22条困境。

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-08-30 DOI: 10.1002/mus.70017

James D Berry, Robert Bowser

引用次数: 0

Aging Accelerates Degradation of Human Neuromuscular Junction Following Peripheral Nerve Injury. 衰老加速周围神经损伤后人类神经肌肉连接处的退化。

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-08-14 DOI: 10.1002/mus.70003

Luigi P Gonzales, Vivian Y Chen, Amanda Tedesco, Saman Andalib, Chris Lee, David J Wright, Tyler R Johnston, Tahseen Mozaffar, Oswald Steward, Ranjan Gupta

{"title":"Aging Accelerates Degradation of Human Neuromuscular Junction Following Peripheral Nerve Injury.","authors":"Luigi P Gonzales, Vivian Y Chen, Amanda Tedesco, Saman Andalib, Chris Lee, David J Wright, Tyler R Johnston, Tahseen Mozaffar, Oswald Steward, Ranjan Gupta","doi":"10.1002/mus.70003","DOIUrl":"https://doi.org/10.1002/mus.70003","url":null,"abstract":"Introduction/aims: Clinical observations indicate differences between young versus older adults in outcomes of peripheral nerve injury (PNI). Here, we present an analysis of motor endplates (MEPs) from normal and denervated deltoid muscle biopsies from young adult (≤ 60 years) and older (> 60 years) patients.Methods: Under an IRB-approved prospective cohort study protocol, 24 patients who underwent open surgery for either shoulder pathology or deltoid dysfunction secondary to axillary nerve injury, confirmed with electrodiagnostic studies, were evaluated. Deltoid muscle biopsies were procured at the time of surgery to assess MEP morphology and innervation status.Results: Analysis of the MEPs in denervated deltoid revealed no significant difference in the percentage of healthy (pretzel) versus unhealthy (intermediate/plaque) morphology between the young and older groups. However, there was a large difference between young versus old denervated deltoids in the percent of innervated MEPs. In young denervated muscle, an average of 48.61% (±12.40%) MEPs were innervated, while in older muscle samples an average of 24.78% (±5.83%) MEPs were innervated. Additionally, the older group had a faster initial rate of MEP nerve signal loss compared to the young group.Discussion: Our study shows that age does affect the MEP response to injury. This effect is most significant in MEP innervation, with rapid loss of visible innervated terminals in the older group. Surprisingly, there was no significant difference in MEP morphology in the older patients, suggesting that early surgical intervention may still offer significant recovery potential.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":"72 5","pages":"1161-1167"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145302059","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}

引用次数: 0

Minimal Clinically Important Differences in Measuring Treatment Effects in CIDP: History, Current Use, Limitations, and Prospects. 测量CIDP治疗效果的最小临床重要差异：历史，当前使用，局限性和前景。

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-07-25 DOI: 10.1002/mus.28478

Yusuf A Rajabally, Kabir K Nazeer, Young Gi Min

{"title":"Minimal Clinically Important Differences in Measuring Treatment Effects in CIDP: History, Current Use, Limitations, and Prospects.","authors":"Yusuf A Rajabally, Kabir K Nazeer, Young Gi Min","doi":"10.1002/mus.28478","DOIUrl":"10.1002/mus.28478","url":null,"abstract":"Outcome measures are essential for evaluating treatment effects and disease progression in chronic inflammatory demyelinating polyneuropathy (CIDP). The concept of the minimal clinically important difference (MCID), which represents the smallest change in a measure deemed clinically meaningful, has become increasingly important in CIDP research, and is also gaining interest in clinical practice. This review explores the history of use in clinical trials and observational studies, as well as potential limitations and future perspectives for MCIDs in CIDP. MCID derivation methods include anchor-based approaches that rely on patient perspectives, and distribution-based methods that calculate the magnitude of changes exceeding statistical error margins. Both approaches have been used in CIDP, yielding MCID cut-offs for key scales such as the Inflammatory Neuropathy Cause and Treatment (INCAT) Scale, Overall Neuropathy Limitation Scale (ONLS), Rasch-built Overall Disability Scale (I-RODS), grip strength, and the Medical Research Council sum score. Challenges include discrepancies in MCID thresholds, particularly for I-RODS and strength measures, and variability related to disease severity and subtype. Despite these issues, MCIDs for disability measures such as INCAT, ONLS, and I-RODS have demonstrated their value through validity and clinical relevance, making them suitable for both research and clinical practice. MCIDs for strength scores, walking tests, sensory scales, and electrophysiological measures lack reliability and direct clinical relevance with regard to the primary concept of clinically meaningful benefit. Future research should focus on optimization of outcome measures, harmonization of MCID derivation methods, and exploration of MCID application with disease-specific Health Related-Quality of Life measures for CIDP.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1042-1051"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12529031/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144718213","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}

引用次数: 0

Reply to the Letter to the Editor: The Role of Patient-Reported Symptoms in Standardizing MGFA Class 0 and MGFA-PIS. 给编辑的回信：患者报告症状在MGFA 0级和MGFA- pis标准化中的作用。

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-09-22 DOI: 10.1002/mus.70027

Katherine Ruzhansky, Yuebing Li, Gil I Wolfe, Srikanth Muppidi, Jeffrey T Guptill, Michael K Hehir, Mazen M Dimachkie, Henry J Kaminski, James F Howard, Pushpa Narayanaswami

引用次数: 0

T2 Hyperintensities in Gracile Tracts of Cervical Spinal Cord in Giant Axonal Neuropathy (GAN). 巨轴索神经病颈脊髓细束的T2高信号。

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-08-16 DOI: 10.1002/mus.70004

Diane Armao, Thomas W Bouldin, Diana X Bharucha-Goebel, Terry S Hartman, Steven J Gray, Rachel M Bailey, Dimah Saade, Joshua J Todd, Minal Jain, Melissa Waite, Carsten G Bönnemann, J Keith Smith

{"title":"T2 Hyperintensities in Gracile Tracts of Cervical Spinal Cord in Giant Axonal Neuropathy (GAN).","authors":"Diane Armao, Thomas W Bouldin, Diana X Bharucha-Goebel, Terry S Hartman, Steven J Gray, Rachel M Bailey, Dimah Saade, Joshua J Todd, Minal Jain, Melissa Waite, Carsten G Bönnemann, J Keith Smith","doi":"10.1002/mus.70004","DOIUrl":"10.1002/mus.70004","url":null,"abstract":"Introduction/aims: Giant axonal neuropathy (GAN) is a hereditary neurodegenerative disease due to the absence or loss of function of the gigaxonin gene. Pathologic findings in GAN are those of \"dying-back\" axonal degeneration, in which the distal axon degenerates but the more proximal axon and neuronal cell body remain intact. Aims of this study were to (1) document imaging abnormalities that may occur in the spinal cords of GAN patients; and (2) assess histologically the spinal cords of GAN rodent models.Methods: A clinical trial of intrathecal (IT) scAAV9/JeT- GAN gene transfer provided a cohort of GAN patients for study. We examined spinal magnetic resonance imaging (MRI) studies from a subset of pretreatment GAN patients ages 6-14 years. For radiologic-pathologic correlation, we examined histologically spinal cords from GAN rodent models with pathological features of human GAN.Results: Of 10 GAN-patient spinal MRIs, 7 showed cervical or diffuse cord atrophy. Five MRIs additionally showed hyperintense, T2-signal abnormalities bilaterally in the cervical gracile tracts. Microscopy of GAN-rodent spinal cords revealed many actively degenerating axons in the cervical gracile tracts but few degenerating axons elsewhere in the cord.Discussion: The localization of spinal T-2 signal abnormalities to the cervical gracile tracts in GAN patients mirrors the localization of active dying-back axonal degeneration in GAN rodent models and suggests that these T2-signal abnormalities may be used as a surrogate marker of active axonal degeneration in the long tracts of the spinal cord in GAN and possibly other dying-back neurodegenerative diseases involving the spinal cord.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1152-1155"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12529028/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144961669","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}

引用次数: 0

The Role of Patient-Reported Symptoms in Standardizing MGFA Class 0 and MGFA-PIS. 患者报告症状在MGFA 0级和MGFA- pis标准化中的作用

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-07-25 DOI: 10.1002/mus.28484

Hai-Feng Li, Chongbo Zhao

引用次数: 0

Association of Race and Social Determinants of Health With Exacerbations in Generalized Myasthenia Gravis. 种族和社会因素与全身性重症肌无力加重的关系

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-09-12 DOI: 10.1002/mus.70022

Louis Jackson, Zhiwen Liu, Jacqueline Pesa, Alicia K Campbell, Ashley E L Anderson

{"title":"Association of Race and Social Determinants of Health With Exacerbations in Generalized Myasthenia Gravis.","authors":"Louis Jackson, Zhiwen Liu, Jacqueline Pesa, Alicia K Campbell, Ashley E L Anderson","doi":"10.1002/mus.70022","DOIUrl":"10.1002/mus.70022","url":null,"abstract":"Introduction/aims: While racial/ethnic disparities exist across many disease states, their influence on the risk of adverse clinical outcomes in large populations of generalized myasthenia gravis (gMG) patients has not been extensively studied. This study assessed the association between race/ethnicity and MG exacerbation risk and examined the potential role of social determinants of health (SDOH).Methods: This retrospective cohort study utilized data from HealthVerity; a large, de-identified, US insurance claims database. Eligible patients were adults with gMG (≥ 2 MG diagnosis code claims, excluding patients with only ophthalmologic specialist visits on/before index date). Inverse probability of treatment weighting using baseline variables was constructed with multinomial distribution for race/ethnicity (Asian/Black/Hispanic/White). Incidence rate ratio (IRR) for ≥ 1 MG exacerbation (defined as an inpatient episode with MG as admitting diagnosis) during follow-up was assessed using a generalized estimating equations model, using \"White\" as the reference. A subset of patients was linked with SDOH variables by residential ZIP code (income-per-capita and percentage of population without health insurance coverage) using the Agency for Healthcare Research and Quality database, and MG exacerbation risk adjusted.Results: Of 10,981 gMG patients, 5147 had linked SDOH data. Weighted risk of MG exacerbations was significantly higher among Black versus White patients (IRR 1.21; 95% confidence interval 1.05-1.39). The association remained after controlling for income and/or health insurance coverage.Discussion: Potential racial disparities exist in the occurrence of MG exacerbations. Further investigation of potential drivers is warranted. At-risk populations should be a focus for healthcare and patient support provision.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1143-1151"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145040930","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}

引用次数: 0

Hearing Loss, Retinal Abnormality, and Seizures in People With Facioscapulohumeral Muscular Dystrophy. 面肩肱骨肌萎缩症患者的听力损失、视网膜异常和癫痫发作。

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-08-20 DOI: 10.1002/mus.70007

Shannon N Kilburn, Shiny Thomas, Anne L Havlik, Katherine D Mathews, Natalie Street, Aida Soim

{"title":"Hearing Loss, Retinal Abnormality, and Seizures in People With Facioscapulohumeral Muscular Dystrophy.","authors":"Shannon N Kilburn, Shiny Thomas, Anne L Havlik, Katherine D Mathews, Natalie Street, Aida Soim","doi":"10.1002/mus.70007","DOIUrl":"10.1002/mus.70007","url":null,"abstract":"Introduction/aims: Few studies describing comorbidities in individuals with facioscapulohumeral muscular dystrophy (FSHD) are available. We used data from the Muscular Dystrophy Surveillance, Tracking and Research Network (MD STARnet) to identify and describe the prevalence of three comorbidities-hearing loss, retinal abnormalities, and seizures-in individuals with FSHD.Methods: We analyzed retrospective population-based data from 548 individuals diagnosed with FSHD who had at least one health visit during 2008-2019. The primary variables of interest were the presence of one or more of the three comorbidities and the age at diagnosis of the comorbidity. We calculated percentages of each comorbidity by population characteristics.Results: Among the study cohort, 17.2% (n = 94) had at least one comorbidity, with 1.5% (n = 8) having multiple comorbidities. Hearing loss (13%; n = 71) was the most frequently reported comorbidity, followed by retinal abnormalities (3.6%; n = 20) and seizures (2.0%; n = 11). Median age at diagnosis for hearing loss, retinal abnormalities, and seizures was 46.5 [interquartile range (IQR):11.8-65.3 years], 58.7 (IQR: 41.5-66.5 years), and 16.5 years (IQR: 3.0-34.7 years), respectively.Discussion: This study demonstrated that a substantial minority of the study cohort had hearing loss, while fewer had retinal abnormalities and seizures. Age at diagnosis varied widely; hearing loss and retinal disease tended to occur in adults, while for seizures, half were ≤ 10 years old. Our results on the prevalence of comorbid conditions among individuals living with FSHD help provide a better understanding of disease burden and support recommendations for ophthalmological and hearing screenings.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1156-1160"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144962225","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}

引用次数: 0

Patients' and Caregivers' Perceptions of Specialty Palliative Care for Amyotrophic Lateral Sclerosis: A Multicenter Evaluation. 肌萎缩性侧索硬化症患者和护理人员对专业姑息治疗的看法：一项多中心评估。

IF 3.1 3区医学

Muscle & Nerve Pub Date : 2025-11-01 Epub Date: 2025-08-19 DOI: 10.1002/mus.70006

Ambereen K Mehta, Gayle Kojimoto, David L O'Riordan, Yaowaree L Leavell, Samuel Maiser, Astrid Grouls, Alexander K Smith, Steven Z Pantilat, Benzi M Kluger, Kara E Bischoff

{"title":"Patients' and Caregivers' Perceptions of Specialty Palliative Care for Amyotrophic Lateral Sclerosis: A Multicenter Evaluation.","authors":"Ambereen K Mehta, Gayle Kojimoto, David L O'Riordan, Yaowaree L Leavell, Samuel Maiser, Astrid Grouls, Alexander K Smith, Steven Z Pantilat, Benzi M Kluger, Kara E Bischoff","doi":"10.1002/mus.70006","DOIUrl":"10.1002/mus.70006","url":null,"abstract":"Introduction/aims: Specialty palliative care (SPC) aims to optimize quality of life for people with life-limiting illnesses. Previous studies support benefits of SPC for people with amyotrophic lateral sclerosis (pALS) and their caregivers; however, few studies have compared patient and caregiver experiences with ALS care and SPC.Methods: An online survey assessing satisfaction with care was distributed to pALS and caregivers who had received SPC. Those who completed the survey were also invited to participate in a semi-structured interview.Results: Thirty-four people (10 pALS, 25 caregivers) from 26 ALS clinics in 20 states completed the survey. Sixteen also provided a qualitative interview. Respondents were most commonly satisfied with their ALS team's ability to answer questions (81%), counsel them regarding ALS medications (78%), and manage motor symptoms (77%). Respondents were least commonly satisfied with their ALS team's ability to manage mood (50%), address spiritual/existential concerns (48%), and help them find in-home care providers (38%). SPC was initiated an average of 14.6 months after ALS diagnosis. Respondents were most commonly satisfied with their SPC team's responsiveness to time-sensitive needs between scheduled visits (82%) and management of mood (80%) and non-pain physical symptoms (73%). Respondents were least commonly satisfied with their SPC team's ability to help with equipment needs (52%), make decisions about procedures (50%), and find in-home caregivers (46%). Interviews highlighted the emotional support and care coordination that SPC teams provide.Discussion: pALS and caregivers identified distinct and complementary strengths of ALS and SPC teams, suggesting that collaboration between teams may provide the most comprehensive care.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1081-1090"},"PeriodicalIF":3.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144874138","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}

引用次数: 0