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Revisiting the Extrapolated Reference Values (E-Ref) Estimation. 重新审视外推参考值(E-Ref)估计。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-10-01 Epub Date: 2026-08-02 DOI: 10.1002/mus.70369
Sanjeev D Nandedkar, Paul E Barkhaus
{"title":"Revisiting the Extrapolated Reference Values (E-Ref) Estimation.","authors":"Sanjeev D Nandedkar, Paul E Barkhaus","doi":"10.1002/mus.70369","DOIUrl":"10.1002/mus.70369","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Extrapolated reference values (E-Ref) is a method to extract reference values (RVs) from a mixed data set (i.e., a data set containing normal and abnormal findings). This is an attractive way for any laboratory to develop their own RVs. Since its original description, we have made enhancements to the E-Ref method. Due to growing interest in this methodology, we describe our current version of the algorithm and interpretation of its results.</p><p><strong>Methods: </strong>The cumulative distribution function (CDF) is the foundation of E-Ref methodology. Computer simulations were used to generate \"normal\" and mixed data sets of conduction velocity. The E-Ref algorithm was revised based on the analysis of CDF in the simulation studies. Bootstrap analysis was performed to assess the variability of estimates and finalize the RV. Median motor nerve conduction parameters from patient studies were analyzed.</p><p><strong>Results: </strong>The CDF shows a \"plateau\" that contains the majority of the normal data even in a mixed data set. The mean of data in the plateau approximated the mean of \"normal\" measurements. The difference between the maximum and minimum in the plateau was close to two standard deviations (SDs).</p><p><strong>Discussion: </strong>The new algorithm establishes three ranges to define findings: definitely normal, definitely abnormal, and borderline. This is consistent with the customary clinical thought process where abnormalities are not \"black and white.\" Bootstrap analysis must be performed for reliable estimates. This may be useful to assess the adequacy of the sample size.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1230-1237"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549155/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148664031","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
Neuralgic Amyotrophy Following B-Cell Maturation Antigen-Directed CAR-T Therapy. b细胞成熟抗原导向CAR-T治疗后神经痛性肌萎缩。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-10-01 Epub Date: 2026-07-30 DOI: 10.1002/mus.70358
Eduardo J Pérez-Torres, Daria V Madeeva, Adam D Cohen, Alfred L Garfall, Chafic Karam
{"title":"Neuralgic Amyotrophy Following B-Cell Maturation Antigen-Directed CAR-T Therapy.","authors":"Eduardo J Pérez-Torres, Daria V Madeeva, Adam D Cohen, Alfred L Garfall, Chafic Karam","doi":"10.1002/mus.70358","DOIUrl":"10.1002/mus.70358","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Chimeric antigen receptor T-cell (CAR-T) therapy has transformed the management of refractory hematologic malignancies, but neurologic complications remain a major concern. While immune effector cell-associated neurotoxicity syndrome is well recognized, peripheral neuropathies such as neuralgic amyotrophy have rarely been described.</p><p><strong>Methods: </strong>We characterize the clinical, electrophysiologic, and imaging features of neuralgic amyotrophy after B-cell maturation antigen (BCMA)-directed CAR-T therapy at a single tertiary care center.</p><p><strong>Results: </strong>We identified four patients with neuralgic amyotrophy following BCMA-directed CAR-T therapy, corresponding to 1.5% of total patients treated in that time. Patients developed acute bilateral shoulder pain followed by weakness 8-12 days after CAR-T infusion. Manifestations included scapular winging and arm weakness, with electromyography showing denervation most prominently in the muscles supplied by the long thoracic and suprascapular nerves. Corticosteroid use appeared to be associated with symptomatic improvement, although residual weakness persisted in most. Diagnosis was delayed in three patients due to misattribution of symptoms to other causes.</p><p><strong>Discussion: </strong>Neuralgic amyotrophy may represent an underrecognized complication of BCMA-directed CAR-T therapy. The consistent timing of onset, bilateral involvement, and responsiveness to corticosteroids support an inflammatory mechanism. Clinicians should maintain vigilance for this complication, as prompt recognition and treatment may mitigate long-term neurological morbidity.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"1275-1280"},"PeriodicalIF":2.9,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13549154/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148630920","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
Systematic Review of Patient- and Observer-Reported Outcomes of Fatigue in Pediatric Neuromuscular Disorders. 儿童神经肌肉疾病患者和观察者报告的疲劳结果的系统评价。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-09-30 DOI: 10.1002/mus.70416
Monica Marzouk, Kayla M D Cornett, Gabrielle A Donlevy, Carl Young, Joshua Burns, Manoj P Menezes, Marnee J McKay
{"title":"Systematic Review of Patient- and Observer-Reported Outcomes of Fatigue in Pediatric Neuromuscular Disorders.","authors":"Monica Marzouk, Kayla M D Cornett, Gabrielle A Donlevy, Carl Young, Joshua Burns, Manoj P Menezes, Marnee J McKay","doi":"10.1002/mus.70416","DOIUrl":"https://doi.org/10.1002/mus.70416","url":null,"abstract":"<p><p>Fatigue is a burdensome symptom for pediatric populations with neuromuscular disorders, often assessed using patient- and observer-reported outcomes. The three aims of this systematic review are to: (1) explore, summarize and critically appraise primary studies that measure fatigue in pediatric neuromuscular disorders using patient- and observer-reported outcomes; (2) compare definitions of fatigue and related terms presented within these studies; and (3) summarize evidence for validation of patient- and observer-reported outcomes used in included studies. Six electronic databases were searched. Of 6801 studies identified, 64 met the inclusion criteria of measuring fatigue with a patient- and/or observer-reported outcome and investigated populations below 22 years of age with neuromuscular disorders. Fatigue assessments included questionnaires (80%), interviews/testimonials (17%), and single-item measures (3%). To measure fatigue, 50% of studies used 1 questionnaire, 28% used 2 or more questionnaires, 6% used interviews alone, 3% used single-item measures alone, and 13% used a combination of these methods. Quality appraisal indicated 80% of included studies had moderate-high risk of bias. Of all included studies, 10 employed varying definitions of fatigue or related terms. Fatigue was commonly described as an overwhelming, multidimensional and whole-body experience. Several questionnaires used to measure fatigue were developed to measure other constructs such as quality of life. Evidence for psychometric validation of all questionnaires was limited, and evidence for validation in neuromuscular disorders and pediatric populations was scarce. This review indicates a need for coherent and standardized fatigue definitions and measures while highlighting the scarcity of evidence for validation of frequently used questionnaires.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149081492","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
Chronic Non-Progressive Neurophysiology in an ALS Patient Treated With Tofersen for 6 Years. 一名接受托佛森治疗6年的ALS患者的慢性非进行性神经生理。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-09-25 DOI: 10.1002/mus.70417
Ivar Winroth, Christian Krarup, Henrik Berntsson, Lisette Salvesen, Kirsten Svenstrup, Erik Nordh, Peter M Andersen
{"title":"Chronic Non-Progressive Neurophysiology in an ALS Patient Treated With Tofersen for 6 Years.","authors":"Ivar Winroth, Christian Krarup, Henrik Berntsson, Lisette Salvesen, Kirsten Svenstrup, Erik Nordh, Peter M Andersen","doi":"10.1002/mus.70417","DOIUrl":"https://doi.org/10.1002/mus.70417","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149062843","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
Quantitative Ultrasound Analysis of Muscle Reinnervation Following Anterior Interosseous Nerve-To-Ulnar Motor Branch Supercharged End-To-Side Transfer Surgery: A Preliminary Study. 前骨间神经-尺侧运动支增压端侧转移术后肌肉再神经的定量超声分析:初步研究。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-09-25 DOI: 10.1002/mus.70407
Rémy Liechti, Esther Vögelin
{"title":"Quantitative Ultrasound Analysis of Muscle Reinnervation Following Anterior Interosseous Nerve-To-Ulnar Motor Branch Supercharged End-To-Side Transfer Surgery: A Preliminary Study.","authors":"Rémy Liechti, Esther Vögelin","doi":"10.1002/mus.70407","DOIUrl":"10.1002/mus.70407","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Routine assessment of muscle reinnervation primarily relies on clinical and electromyographic evaluation. The aim of this preliminary study was to evaluate sensitivity of quantitative ultrasound analysis for monitoring intrinsic hand muscle recovery following anterior interosseous nerve (AIN)-to-ulnar motor branch supercharged end-to-side (SETS) transfer.</p><p><strong>Methods: </strong>Eight patients (five male, three female; mean age 54.5 years, range 10-80) underwent anterior ulnar nerve transposition with AIN-to-ulnar motor branch SETS transfer. High-resolution ultrasound assessed the first dorsal interosseous (FDI) and hypothenar muscles pre- and postoperatively using quantitative grayscale and cross-sectional thickness (CST) analysis. Functional outcomes included key pinch and grip strength.</p><p><strong>Results: </strong>Preoperatively, affected FDI and hypothenar muscles exhibited significantly higher grayscale and lower CST values compared with the contralateral side. Following surgery, this difference decreased significantly in both muscle groups (FDI: mean difference [MD] -19.9, 95% CI -33.5 to -6.3; p = 0.013; hypothenar: MD -21.2, 95% CI -32.1 to -10.3; p = 0.006). CST measurements improved in the FDI muscle (MD -7.1 mm, 95% CI -11.3 to -2.9; p = 0.009), while there was no significant CST change in the hypothenar muscle (MD -2.3 mm, 95% CI -4.9 to 0.4; p = 0.081). There was a significant correlation between FDI muscle grayscale values and key pinch strength (R<sup>2</sup> = 0.31), whereas hypothenar echogenicity showed no correlation with grip strength (R<sup>2</sup> = 0.0001).</p><p><strong>Discussion: </strong>This preliminary study suggests that quantitative ultrasound of intrinsic hand muscles appears sensitive and can detect structural changes during reinnervation following AIN-to-ulnar motor branch SETS transfer.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149039911","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
Muscle Imaging Patterns and Variant-Specific Progression in DNM2-Related Centronuclear Myopathy. 与dnm2相关的核中性肌病的肌肉成像模式和变异特异性进展。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-09-25 DOI: 10.1002/mus.70411
Genevieve Gucci Grace Capispisan Uy, Satoru Noguchi, Shinichiro Hayashi, Yuji Takahashi, Ichizo Nishino
{"title":"Muscle Imaging Patterns and Variant-Specific Progression in DNM2-Related Centronuclear Myopathy.","authors":"Genevieve Gucci Grace Capispisan Uy, Satoru Noguchi, Shinichiro Hayashi, Yuji Takahashi, Ichizo Nishino","doi":"10.1002/mus.70411","DOIUrl":"https://doi.org/10.1002/mus.70411","url":null,"abstract":"<p><strong>Introduction/aims: </strong>DNM2-related centronuclear myopathy (DNM2-CNM) exhibits characteristic imaging patterns, but imaging-based staging and variant-specific severity pattern remain incompletely defined. We aimed to (1) characterize muscle involvement patterns, (2) establish imaging-based severity stages and identify muscles that best distinguish severity groups, and (3) model variant- and muscle-specific age-related score patterns, with comparison to adult-onset MTM1-related CNM.</p><p><strong>Methods: </strong>We retrospectively analyzed whole-body muscle imaging (computed tomography/magnetic resonance imaging) in 30 genetically confirmed DNM2-CNM patients. Forty-five muscles were scored using the modified Mercuri score (mMs 0-4). Unsupervised uniform manifold approximation and projection (UMAP) and hierarchical clustering defined severity groups and involvement patterns. Violin plots characterized the distribution of mMs scores across severity stages. Linear regression and generalized additive models characterized variant and muscle-specific age-associated mMs patterns. Modal mMs values were used to generate comparative schematics for DNM2-CNM and MTM1-related CNM.</p><p><strong>Results: </strong>UMAP identified three severity groups. Hierarchical clustering revealed a distal-predominant pattern with early severe involvement of posterior calf and paraspinal muscles, followed by posterior thigh and gluteal muscles. Variant modeling demonstrated distinct age-associated mMs patterns: PH domain variants (A618T/S615L/S619L) showed earliest onset and steepest age-associated rate of mMs increase (slope 10.1, p = 0.003), middle-domain variants (E368K/R369G/R369Q) showed intermediate rate of mMs increase (slope 2.86), R465W was milder (slope 2.56), and R369W was slowest (slope 0.74). DNM2-CNM showed distal-to-proximal involvement, contrasting with the proximal-predominant pattern of MTM1-related CNM.</p><p><strong>Discussion: </strong>Muscle imaging defines reproducible severity stages and variant-specific age-associated severity patterns, providing a quantitative imaging framework for DNM2-CNM staging, monitoring, and risk stratification.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149062858","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
Predictors for Successful Non-Invasive Ventilation in Patients With Neuralgic Amyotrophy and Phrenic Nerve Involvement. 神经痛性肌萎缩和膈神经受累患者无创通气成功的预测因素。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-09-24 DOI: 10.1002/mus.70403
A B Hendrikx, R T Sprooten, B A Vosse, S C Steijns, S M van Kuijk, J G Hoeijmakers, F M Franssen
{"title":"Predictors for Successful Non-Invasive Ventilation in Patients With Neuralgic Amyotrophy and Phrenic Nerve Involvement.","authors":"A B Hendrikx, R T Sprooten, B A Vosse, S C Steijns, S M van Kuijk, J G Hoeijmakers, F M Franssen","doi":"10.1002/mus.70403","DOIUrl":"https://doi.org/10.1002/mus.70403","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Phrenic nerve involvement in neuralgic amyotrophy (NA) may cause diaphragm paresis and respiratory impairment, for which chronic non-invasive ventilation (NIV) may be beneficial. Data on treatment success and predictors are lacking. This study evaluated NIV treatment success and its predictors in NA patients with diaphragm paresis.</p><p><strong>Methods: </strong>Clinical data of all NA patients initiated on chronic NIV at the Centre of Home Mechanical Ventilation (Maastricht, the Netherlands) between September 2008 and April 2023 were retrospectively analyzed. Successful NIV use was defined as continued NIV or successful step-down due to clinical improvement. Cox proportional-hazards regression analyses were performed to identify predictors of successful outcome.</p><p><strong>Results: </strong>Fifty-four patients (mean age 62.5 ± 10.9 years; 72.2% male) were included. Mean baseline postural decline in forced vital capacity (ΔFVC) was -35.1% ± 19.6%. Median NIV adherence was 6.4 [3.6-8.1] hours/day. Thirty-eight patients (70.4%) continued NIV during a mean follow-up of 4.9 years. Of these, six (11%) were successfully stepped down from NIV following clinical and/or functional improvement. Sixteen patients discontinued NIV because of lack of effect or intolerance. Baseline ΔFVC (sitting to supine) independently predicted successful NIV use (HR 0.947; 95% CI: 0.91-0.99).</p><p><strong>Discussion: </strong>Chronic NIV was successful in most NA patients with diaphragm paresis, supporting its role as a long-term treatment option. Greater postural decline in FVC predicted treatment success, suggesting that postural spirometry may help identify patients most likely to benefit from NIV.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149039876","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
The Two-Dimensional Framework of Nerve Injury. 神经损伤的二维框架。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-09-24 DOI: 10.1002/mus.70409
Shawn P Jorgensen, Sandra L Hearn, Christopher J Lamb, Lawrence R Robinson
{"title":"The Two-Dimensional Framework of Nerve Injury.","authors":"Shawn P Jorgensen, Sandra L Hearn, Christopher J Lamb, Lawrence R Robinson","doi":"10.1002/mus.70409","DOIUrl":"10.1002/mus.70409","url":null,"abstract":"<p><p>Classifying nerve injury severity is essential for communication, prognosis, and treatment decisions. Traditional classification systems (Seddon, Sunderland) cannot account for the full spectrum of nerve pathology, from familiar phenomena such as local anesthetics to recently discovered autoimmune nodopathies. A two-dimensional framework is proposed that separates two aspects of severity conflated in previous classifications and applies to focal and diffuse nerve injuries. The first dimension, quantitative degree, is the proportion of axons involved; the second, pathological type, is the underlying pathology of the involved axons. Pathological type comprises conduction block (conduction failure in live axons) and axon loss (conduction failure of dead axons). Conduction block has two anatomic origins: axonal block, due to primary axonal dysfunction, and demyelinating block, due to primary myelin dysfunction. Axonal block can be organized into three mechanistic clusters: ionic, displacement, and nodopathic block, with distinct pathophysiology, histology, and recovery times. Both axonal and demyelinating conduction block produce electrodiagnostic features historically attributed to demyelination, although subtle differences can sometimes be identified. Severity and recovery time increase from ionic block to axon loss. A single mechanism can cause multiple pathological types, sometimes simultaneously, with the type determined by injury intensity and duration. The two dimensions integrate into assessment: quantitative degree is estimated clinically, and conduction block or axon loss is determined electrodiagnostically. This framework highlights that conduction block can result from processes at the axon or myelin. This includes autoimmune nodopathies, which are often mistaken for demyelinating disorders, but accurate identification can change treatment and may be lifesaving.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149030575","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
Reference Electrode Position Affects Compound Muscle Action Potential Amplitude in Ulnar Nerve Conduction Studies Recorded From the First Dorsal Interosseous Muscle. 参考电极位置影响从第一背骨间肌记录的尺神经传导研究中复合肌肉动作电位振幅。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-09-20 DOI: 10.1002/mus.70415
Ross Penninkilampi, Catherine Beadon, Con Yiannikas, Matthew Silsby
{"title":"Reference Electrode Position Affects Compound Muscle Action Potential Amplitude in Ulnar Nerve Conduction Studies Recorded From the First Dorsal Interosseous Muscle.","authors":"Ross Penninkilampi, Catherine Beadon, Con Yiannikas, Matthew Silsby","doi":"10.1002/mus.70415","DOIUrl":"https://doi.org/10.1002/mus.70415","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Nerve conduction studies (NCSs) of the ulnar nerve recording from the first dorsal interosseous muscle (FDI) are clinically relevant in assessing ulnar neuropathy and the split-hand index. Reference electrode (E2) position varies across normative datasets, with the base of the thumb favored to avoid an initial positive deflection in the compound muscle action potential (CMAP). While latency effects of E2 position have been described, consequences for CMAP amplitude and area have not been systematically examined in a within-subject comparison. This study aims to evaluate CMAP parameters in paired ulnar motor NCS recordings from FDI with E2 over the base of the index finger or thumb.</p><p><strong>Methods: </strong>This retrospective study analyzed 183 ulnar motor NCS recordings from FDI in 155 patients referred for neurophysiological testing. CMAP amplitude, latency, negative peak duration, and area were recorded with E2 positioned at the base of the thumb and then the index finger. Within-subjects comparison was assessed using paired t-tests, with Bland-Altman analysis to assess agreement.</p><p><strong>Results: </strong>CMAP amplitude was on average 2.8 mV lower with E2 at the thumb than at the index finger (95% CI: -3.1 to -2.4; p < 0.0001), with a corresponding reduction in CMAP area. There was no clinically meaningful effect on latency or peak duration. Bland-Altman analysis showed no proportional bias. In 40% of studies with abnormal CMAP amplitude at the thumb, repositioning E2 to the index finger normalized the amplitude.</p><p><strong>Discussion: </strong>Reference electrode position substantially affects ulnar FDI CMAP amplitude, with implications for ulnar neuropathy diagnosis and split-hand index calculation.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149012800","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
Clinical Outcomes and Treatment Efficacy in Juvenile Myasthenia Gravis: A Retrospective Review From a Single Center. 青少年重症肌无力的临床结局和治疗效果:单中心回顾性分析。
IF 2.9 3区 医学
Muscle & Nerve Pub Date : 2026-09-16 DOI: 10.1002/mus.70408
Tanitnun Paprad, Elisa Nigro, Zahra Aleisa, Freddy Paiz, Hernan Gonorazky
{"title":"Clinical Outcomes and Treatment Efficacy in Juvenile Myasthenia Gravis: A Retrospective Review From a Single Center.","authors":"Tanitnun Paprad, Elisa Nigro, Zahra Aleisa, Freddy Paiz, Hernan Gonorazky","doi":"10.1002/mus.70408","DOIUrl":"https://doi.org/10.1002/mus.70408","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Early recognition and treatment are critical in juvenile myasthenia gravis (JMG), yet pediatric-specific evidence remains limited. We aimed to describe treatment patterns, remission rates, and predictors in a Canadian cohort.</p><p><strong>Methods: </strong>We conducted a retrospective study of patients under 18 years diagnosed with JMG at The Hospital for Sick Children, Toronto, between 2015 and 2025. Clinical, treatment, and outcome data were collected. Diagnosis was confirmed by antibody positivity or electrophysiology, and outcomes were classified using the Myasthenia Gravis Foundation of America postintervention status (MGFA-PIS). Kaplan-Meier and Cox regression analyses were used to evaluate remission and its predictors.</p><p><strong>Results: </strong>Thirty-six patients were included, 16 with generalized MG (GMG) and 20 with ocular MG (OMG). Mean age at diagnosis was 8.7 years; 50% were female. Acetylcholine receptor-positive, muscle-specific kinase-positive, and seronegative MG accounted for 55.6%, 5.6%, and 38.9% of the cohort, respectively. Repetitive nerve stimulation was abnormal in 82.6% tested. Most patients received pyridostigmine and corticosteroids initially, with additional immunosuppressants in selected cases. Seven received rituximab, and seven underwent thymectomy, with most achieving complete stable remission (CSR). The estimated 3-year remission rate was 36.1%, and 66.7% achieved CSR at last follow-up (median 45.5 months). Older age at onset and early immunosuppression predicted remission, while GMG had a higher rate than OMG (56.2% vs. 20.6%, p = 0.044).</p><p><strong>Discussion: </strong>Earlier immunosuppression was associated with a higher remission rate, whereas treatment tended to be initiated later and remission was less frequent in OMG. These findings support a timely treatment approach in JMG.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2026-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148983160","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
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