Muscle & Nerve最新文献

{"title":"Phrenic neuropathy: A different flavor of the same dish.","authors":"Sandra L Hearn, Andrea J Boon","doi":"10.1002/mus.28264","DOIUrl":"https://doi.org/10.1002/mus.28264","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350506","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":"The use of electrical stimulation to enhance recovery following peripheral nerve injury.","authors":"Paige B Hardy, Bonnie Y Wang, K Ming Chan, Christine A Webber, Jenna-Lynn B Senger","doi":"10.1002/mus.28262","DOIUrl":"https://doi.org/10.1002/mus.28262","url":null,"abstract":"<p><p>Peripheral nerve injury is common and can have devastating consequences. In severe cases, functional recovery is often poor despite surgery. This is primarily due to the exceedingly slow rate of nerve regeneration at only 1-3 mm/day. The local environment in the distal nerve stump supportive of nerve regrowth deteriorates over time and the target end organs become atrophic. To overcome these challenges, investigations into treatments capable of accelerating nerve regrowth are of great clinical relevance and are an active area of research. One intervention that has shown great promise is perioperative electrical stimulation. Postoperative stimulation helps to expedite the Wallerian degeneration process and reduces delays caused by staggered regeneration at the site of nerve injury. By contrast, preoperative \"conditioning\" stimulation increases the rate of nerve regrowth along the nerve trunk. Over the past two decades, a rich body of literature has emerged that provides molecular insights into the mechanism by which electrical stimulation impacts nerve regeneration. The end result is upregulation of regeneration-associated genes in the neuronal body and accelerated transport to the axon front for regrowth. The efficacy of brief electrical stimulation on patients with peripheral nerve injuries was demonstrated in a number of randomized controlled trials on compressive, transection and traction injuries. As approved equipment to deliver this treatment is becoming available, it may be feasible to deploy this novel treatment in a wide range of clinical settings.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350507","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":"Development of an ultrasound-based metric of muscle functional capacity for use in patients with neuromuscular disease.","authors":"Allison N McCrady, Chelsea D Masterson, Laura E Barnes, Rebecca J Scharf, Silvia S Blemker","doi":"10.1002/mus.28263","DOIUrl":"https://doi.org/10.1002/mus.28263","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Spinal muscular atrophy (SMA) and Duchenne muscular dystrophy (DMD) are progressive neuromuscular disorders characterized by severe muscle weakness and functional decline (Pillen et al., Muscle Nerve 2008; 37(6):679-693). With new therapeutics, objective methods with increased sensitivity are needed to assess muscle function. Ultrasound imaging is a promising approach for assessing muscle fat and fibrosis in neuromuscular disorders. This study builds on prior work by combining ultrasound-based measurements of muscle size, shape, and quality, relating these measures to muscle strength, and proposing a multivariable image-based estimate of muscle function.</p><p><strong>Methods: </strong>Maximum voluntary elbow flexion torque of 36 participants (SMA, DMD, and healthy controls) was measured by hand-held dynamometry and elbow flexor muscles were imaged using ultrasound. Muscle size (cross-sectional area, maximum Feret diameter or width, and thickness), quality (echogenicity, texture anisotropy index), and cross-sectional shape (diameter ratio) were measured. Multivariable regression was used to select ultrasound measurements that predict elbow flexion torque.</p><p><strong>Results: </strong>Significant differences were observed in muscle size (decreased), shape (thinned), and quality (decreased) with increased disease severity and compared to healthy participants. CSA (brachioradialis R² = 0.51), maximum Feret diameter (biceps R² = 0.49, brachioradialis R² = 0.58) and echogenicity (brachioradialis R² = 0.61) were most correlated with torque production. Multivariable regression models identified that muscle size (CSA, maximum Feret diameter) and quality (echogenicity) were both essential to predict elbow flexion torque (R² = 0.65).</p><p><strong>Discussion: </strong>A multivariable approach combining muscle size and quality improves strength predictions over single variable approaches. These methods present a promising avenue for the development of sensitive and functionally relevant biomarkers of neuromuscular disease.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350504","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":"Anatomical validation of needle placement for cervical paraspinal mapping of the multifidus.","authors":"Brian Malave, Steven Johnson, Andrew J Haig","doi":"10.1002/mus.28268","DOIUrl":"https://doi.org/10.1002/mus.28268","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The paraspinal muscles are an important component of electrodiagnostic testing for radiculopathy. In the cervical region, a protocol for cervical paraspinal mapping assumes accurate placement of the electromyography (EMG) needle into the cervical multifidus. However, there is scant information regarding the accuracy of needle placement in that muscle. This study examines the accuracy of this protocol in sampling the intended multifidus muscle of cadavers.</p><p><strong>Methods: </strong>An experienced electromyographer directed needles to multifidus at C5, C7, and T2 spinous processes of 19 embalmed cadavers, and injected color dyes. Separately another examiner dissected the cadavers, noting whether the dye was in the correct location and whether its trajectory approached any \"danger zones\" of nerve, artery, or joint.</p><p><strong>Results: </strong>The dye was in the multifidus muscle 100% of the time and hit the intended bony target in 79%, 73%, and 79% of C5, C7, and T2 insertions. No insertion was found in a danger zone.</p><p><strong>Discussion: </strong>The results indicate modest accuracy for EMG needle placement as proposed in the clinical protocol. Clinical utility of this method requires more work including the establishment of norms, sensitivities, specificities, and clinical impact.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350503","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":"Intravenous immunoglobulin and plasma exchange prescribing patterns for Guillain-Barre Syndrome in the United States-2001 to 2018.","authors":"Amro M Stino, Evan L Reynolds, Maya Watanabe, Brian C Callaghan","doi":"10.1002/mus.28265","DOIUrl":"https://doi.org/10.1002/mus.28265","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Randomized controlled trials show that repeat intravenous immunoglobulin (IVIG) dosing and plasma exchange (PLEX) followed by IVIG (combination therapy) have no additional therapeutic benefit in Guillain-Barre Syndrome (GBS) non-responders. Furthermore, the delineation between GBS and Acute Onset CIDP (A-CIDP) can be particularly challenging and carries therapeutic implications. We aimed to evaluate the presence of repeat IVIG, combination therapy, and diagnostic reclassification from GBS to CIDP.</p><p><strong>Methods: </strong>We performed a retrospective study of a large healthcare database for patients with GBS in the US from 2001 to 2018. We identified individuals initially diagnosed with GBS and later re-classified as CIDP. Multivariable logistic regression models were developed to determine associations between patient factors and repeat IVIG dosing, combination therapy, and diagnostic re-classification from GBS to CIDP.</p><p><strong>Results: </strong>We identified 2325 patients with GBS. A total of 39.7% received repeat IVIG and 6.1% received combination therapy. The proportion of individuals initially diagnosed with GBS and then re-classified as CIDP was 32.0%. Repeat IVIG, combination therapy, and diagnostic reclassification remained stable over time. Female sex (OR 0.79, 95% CI 0.65-0.96) and medium-high net worth (OR 0.64, 95% CI 0.45-0.90) associated with repeat IVIG therapy, while Asian ethnicity associated with diagnostic re-classification from GBS to CIDP (OR 1.77, 95% CI 1.09-2.86).</p><p><strong>Discussion: </strong>Repeat IVIG dosing was quite common in GBS before newer trials suggesting harm in non-responders, and IVIG/PLEX combination therapy continues to persist despite strong evidence against use in non-responders. Further, nearly one in three patients initially diagnosed with GBS is subsequently diagnosed with CIDP, but the reasons are unclear.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350505","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":"Volumetric muscle composition analysis in sporadic inclusion body myositis using fat-referenced magnetic resonance imaging: Disease pattern, repeatability, and natural progression.","authors":"John Heerfordt, Markus Karlsson, Midori Kusama, Seiya Ogata, Ryuta Mukasa, Naoki Kiyosawa, Noriko Sato, Per Widholm, Olof Dahlqvist Leinhard, André Ahlgren, Madoka Mori-Yoshimura","doi":"10.1002/mus.28252","DOIUrl":"https://doi.org/10.1002/mus.28252","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Fat-referenced magnetic resonance imaging (MRI) has emerged as a promising volumetric technique for measuring muscular volume and fat in neuromuscular disorders, but the experience in inflammatory myopathies remains limited. Therefore, this work aimed at describing how sporadic inclusion body myositis (sIBM) manifests on standardized volumetric fat-referenced MRI muscle measurements, including within-scanner repeatability, natural progression rate, and relationship to clinical parameters.</p><p><strong>Methods: </strong>Ten sIBM patients underwent whole-leg Dixon MRI at baseline (test-retest) and after 12 months. The lean muscle volume (LMV), muscle fat fraction (MFF), and muscle fat infiltration (MFI) of the quadriceps, hamstrings, adductors, medial gastrocnemius, and tibialis anterior were computed. Clinical assessments of IBM Functional Rating Scale (IBMFRS) and knee extension strength were also performed. The baseline test-retest MRI measurements were used to estimate the within-subject standard deviation (s_w). 12-month changes were derived for all parameters.</p><p><strong>Results: </strong>The MRI measurements showed high repeatability in all muscles; s_w ranged from 2.7 to 18.0 mL for LMV, 0.7-1.3 percentage points (pp) for MFF, and 0.2-0.7 pp for MFI. Over 12 months, average LMV decreased by 7.4% while MFF and MFI increased by 3.8 pp and 1.8 pp, respectively. Mean IBMFRS decreased by 2.4 and mean knee extension strength decreased by 32.8 N.</p><p><strong>Discussion: </strong>The MRI measurements showed high repeatability and 12-month changes consistent with muscle atrophy and fat replacement as well as a decrease in both muscle strength and IBMFRS. Our findings suggest that fat-referenced MRI measurements are suitable for assessing disease progression and treatment response in inflammatory myopathies.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":2.8,"publicationDate":"2024-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142350508","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":"Test-retest and inter-rater reliability of two devices measuring tactile mechanical detection thresholds in healthy adults: Semmes-Weinstein monofilaments and the cutaneous mechanical stimulator.","authors":"Elisa Mamino,Ségolène Lithfous,Thierry Pebayle,André Dufour,Olivier Després","doi":"10.1002/mus.28258","DOIUrl":"https://doi.org/10.1002/mus.28258","url":null,"abstract":"INTRODUCTION/AIMSLimitations exist in evaluating mechanical detection thresholds (MDTs) due to a lack of dependable electronic instruments designed to assess Aβ fibers and measure MDTs across different body areas. This study aims to evaluate the test-retest and inter-rater reliability of the cutaneous mechanical stimulator (CMS), an electronic tactile stimulator, in quantifying MDTs.METHODSUsing a test-retest design, participants underwent assessments of MDTs using Semmes-Weinstein monofilaments (SWM) and the CMS. This study included 27 healthy volunteers (mean age 24.07 ± 3.76 years). Two raters assessed MDTs using SWM and the CMS at two stimulation sites (the left hand and foot) in two experimental sessions approximately 2 weeks apart.RESULTSMDTs using SWM and the CMS showed excellent reliability on the hand (intraclass correlation coefficient [ICC] = .84) and foot (ICC = .90). A comparison of results obtained at the two sessions showed that MDTs on the hand displayed good reliability for both SWM (ICC = .63) and the CMS (ICC = .73), whereas MDTs on the foot displayed fair reliability for SWM (ICC = .50) and the CMS (ICC = .42). MDTs exhibited good inter-rater reliability with SWM (ICC = .66) and excellent inter-rater reliability with the CMS (ICC = .82) on the hand, as well as showing fair inter-rater reliability with SWM (ICC = .53) and good inter-rater reliability with the CMS (ICC = .60) on the foot.DISCUSSIONThe CMS showed superior inter-rater reliability, indicating its potential as a valuable tool for assessing tactile sensitivity in research and clinical settings.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142247270","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":"Lower limb nerve ultrasound: A four-way comparison of acquired and inherited axonopathy, inherited neuronopathy and healthy controls.","authors":"Luciana Pelosi,Daniele Coraci,Eoin Mulroy,Ruth Leadbetter,Luca Padua,Richard Roxburgh","doi":"10.1002/mus.28260","DOIUrl":"https://doi.org/10.1002/mus.28260","url":null,"abstract":"INTRODUCTION/AIMSIn a recent study, we showed that nerve ultrasound of the upper limbs could distinguish inherited sensory neuronopathy from inherited axonopathy; surprisingly, no differences were found in the lower limb nerves. In this study, we compared lower limb nerve ultrasound measurements in inherited neuronopathy, inherited axonopathy, and acquired axonopathy.METHODSTibial and sural nerve ultrasound cross-sectional areas (CSAs) of 34 healthy controls were retrospectively compared with those of three patient groups: 17 with cerebellar ataxia with neuronopathy and vestibular areflexia syndrome (CANVAS), 18 with Charcot-Marie-Tooth type 2 (CMT2), and 18 with acquired length-dependent sensorimotor axonal neuropathy, using ANOVA with post-hoc Tukey honestly significance difference (HSD) (significance level set at p < .05).RESULTSThe nerve CSAs of CANVAS and CMT2 patients were not significantly different. Both the tibial and the sural nerve CSAs were significantly smaller in CANVAS and CMT2 compared with the acquired axonal neuropathy group. Tibial nerve CSAs of CANVAS and CMT2 were significantly smaller than controls. Tibial and sural nerve CSAs of the acquired axonal neuropathy group were also significantly larger than the controls'.DISCUSSIONUltrasound of the lower limb nerves distinguished inherited from acquired axonopathy with the nerve size respectively reduced and increased in these two groups. This has potential implication for the differential diagnosis of these diseases in clinical practice.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142247269","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":"Neuroma morphology: A macroscopic classification system.","authors":"Floris V Raasveld,Daniel T Weigel,Wen-Chih Liu,Maximilian Mayrhofer-Schmid,Barbara Gomez-Eslava,Vlad Tereshenko,Charles D Hwang,Brian J Wainger,William Renthal,Mark Fleming,Ian L Valerio,Kyle R Eberlin","doi":"10.1002/mus.28261","DOIUrl":"https://doi.org/10.1002/mus.28261","url":null,"abstract":"INTRODUCTION/AIMSNeuromas come in different shapes and sizes; yet the correlation between neuroma morphology and symptomatology is unknown. Therefore, we aim to investigate macroscopic traits of excised human neuromas and assess the validity of a morphological classification system and its potential clinical implications.METHODSEnd-neuroma specimens were collected from prospectively enrolled patients undergoing symptomatic neuroma surgery. Protocolized images of the specimens were obtained intraoperatively. Pain data (Numeric rating scale, 0-10) were prospectively collected during preoperative interview, patient demographic and comorbidity factors were collected from chart review. A morphological classification is proposed, and the inter-rater reliability (IRR) was assessed. Distribution of neuroma morphology with patient factors, was described.RESULTSForty-five terminal neuroma specimens from 27 patients were included. Residual limb patients comprised 93% of the population, of which 2 were upper (8.0%) and 23 (92.0%) were lower extremity residual limb patients. The proposed morphological classification, consisting of three groups (bulbous, fusiform, atypical), demonstrated a strong IRR (Cohen's kappa = 0.8). Atypical neuromas demonstrated higher preoperative pain, compared with bulbous and fusiform. Atypical morphology was more prevalent in patients with diabetes and peripheral vascular disease.DISCUSSIONA validated morphological classification of neuroma is introduced. These findings may assist surgeons and researchers with better understanding of symptomatic neuroma development and their clinical implications. The potential relationship of neuroma morphology with the vascular and metabolic microenvironment requires further investigation.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142247533","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":"Access for ALL in ALS: A large-scale, inclusive, collaborative consortium to unlock the molecular and genetic mechanisms of amyotrophic lateral sclerosis.","authors":"James D Berry,Sabrina Paganoni,Matthew B Harms,Neil Shneider,Jinsy Andrews,Timothy M Miller,Suma Babu,Alex V Sherman,Brent T Harris,Frank A Provenzano,Hemali P Phatnani,Jeremy Shefner,Mark A Garret,Shaffeeq S Ladha,Amy Y Tsou,Praveena Mohan,Courtney Igne,,Robert Bowser","doi":"10.1002/mus.28244","DOIUrl":"https://doi.org/10.1002/mus.28244","url":null,"abstract":"Recent progress in therapeutics for amyotrophic lateral sclerosis (ALS) has spurred development and imbued the field of ALS with hope for more breakthroughs, yet substantial scientific gaps persist. This unmet need remains a stark reminder that innovative paradigms are needed to invigorate ALS research. To move toward more informative, targeted, and personalized drug development, the National Institutes of Health (NIH) established a national ALS clinical research consortium called Access for ALL in ALS (ALL ALS). This new consortium is a multi-institutional effort that aims to organize the ALS clinical research landscape in the United States. ALL ALS is operating in partnership with several stakeholders to operationalize the recommendations of the Accelerating Access to Critical Therapies for ALS Act (ACT for ALS) Public Private Partnership. ALL ALS will provide a large-scale, centralized, and readily accessible infrastructure for the collection and storage of a wide range of data from people living with ALS (symptomatic cohort) or who may be at risk of developing ALS (asymptomatic ALS gene carriers). Importantly, ALL ALS is designed to encourage community engagement, equity, and inclusion. The consortium is prioritizing the enrollment of geographically, ethnoculturally, and socioeconomically diverse participants. Collected data include longitudinal clinical data and biofluids, genomic, and digital biomarkers that will be harmonized and linked to the central Accelerating Medicines Partnership for ALS (AMP ALS) portal for sharing with the research community. The aim of ALL ALS is to deliver a comprehensive, inclusive, open-science dataset to help researchers answer important scientific questions of clinical relevance in ALS.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142247271","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}