Muscle & Nerve最新文献

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. 测量健康成年人触觉机械检测阈值的两种设备的测试重复可靠性和评分者之间的可靠性：塞姆斯-温斯坦单丝和皮肤机械刺激器。

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-19 DOI: 10.1002/mus.28258

Elisa Mamino,Ségolène Lithfous,Thierry Pebayle,André Dufour,Olivier Després

{"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}

引用次数: 0

Lower limb nerve ultrasound: A four-way comparison of acquired and inherited axonopathy, inherited neuronopathy and healthy controls. 下肢神经超声：获得性和遗传性轴索病、遗传性神经病和健康对照组的四项比较。

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-19 DOI: 10.1002/mus.28260

Luciana Pelosi,Daniele Coraci,Eoin Mulroy,Ruth Leadbetter,Luca Padua,Richard Roxburgh

{"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}

引用次数: 0

Neuroma morphology: A macroscopic classification system. 神经瘤形态：宏观分类系统

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-19 DOI: 10.1002/mus.28261

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

{"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}

引用次数: 0

Access for ALL in ALS: A large-scale, inclusive, collaborative consortium to unlock the molecular and genetic mechanisms of amyotrophic lateral sclerosis. 肌萎缩性脊髓侧索硬化症（ALS）的全科治疗：一个大规模、包容性的合作联盟，旨在揭示肌萎缩性脊髓侧索硬化症的分子和遗传机制。

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-18 DOI: 10.1002/mus.28244

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

{"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}

引用次数: 0

The impact of genotype on age at loss of ambulation in individuals with Duchenne muscular dystrophy treated with corticosteroids: A single‐center study of 555 patients 基因型对接受皮质类固醇治疗的杜氏肌营养不良症患者丧失行动能力年龄的影响：一项针对 555 名患者的单中心研究

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-18 DOI: 10.1002/mus.28255

Alexander Zygmunt, Brenda Wong, David Moon, Paul Horn, Richard Rathbun, Joshua Lambert, Jean Bange, Irina Rybalsky, Lisa Reebals, Cuixia Tian

{"title":"The impact of genotype on age at loss of ambulation in individuals with Duchenne muscular dystrophy treated with corticosteroids: A single‐center study of 555 patients","authors":"Alexander Zygmunt, Brenda Wong, David Moon, Paul Horn, Richard Rathbun, Joshua Lambert, Jean Bange, Irina Rybalsky, Lisa Reebals, Cuixia Tian","doi":"10.1002/mus.28255","DOIUrl":"https://doi.org/10.1002/mus.28255","url":null,"abstract":"Introduction/AimsStudies have demonstrated that certain genotypes in Duchenne muscular dystrophy (DMD) have milder or more severe phenotypes. These studies included individuals treated and not treated with corticosteroids and multiple sites with potentially varying standards of care. We aimed to assess genotype–phenotype correlations for age at loss of ambulation (LoA) in a large cohort of individuals with DMD treated with corticosteroids at one center.MethodsIn this retrospective review of medical records, encounters were included for individuals diagnosed with DMD if prescribed corticosteroids, defined as daily deflazacort or prednisone or high‐dose weekend prednisone, for 12 consecutive months. Encounters were excluded if the participants were taking disease‐modifying therapy. Data were analyzed using survival analysis for LoA and Fisher's exact tests to assess the percentage of late ambulatory (>14 years old) individuals for selected genotypes.ResultsOverall, 3948 encounters from 555 individuals were included. Survival analysis showed later age at LoA for exon 44 skip amenable (p = .004), deletion exons 3–7 (p < .001) and duplication exon 2 (p = .043) cohorts and earlier age at LoA for the exon 51 skip amenable cohort (p < .001) when compared with the rest of the cohort. Individuals with deletions of exons 3–7 had significantly more late ambulatory individuals than other cohorts (75%), while those with exon 51 skip amenable deletions had significantly fewer (11.9%) compared with other cohorts.DiscussionThis confirms previous observations of genotype–phenotype correlations in DMD and enhances information for trial design and clinical management.","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":"142247272","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

Electrophysiological features of the peripheral neuropathy in patients with pathologic biallelic RFC1 repeat expansions 双倍拷贝 RFC1 重复扩增患者周围神经病变的电生理特征

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-17 DOI: 10.1002/mus.28257

Calezis Claudia, Bonello‐Palot Nathalie, Verschueren Annie, Azulay Jean‐Philippe, Fortanier Etienne, Grapperon Aude‐Marie, Kouton Ludivine, Gallard Julien, Salort‐Campana Emmanuelle, Attarian Shahram, Delmont Emilien

{"title":"Electrophysiological features of the peripheral neuropathy in patients with pathologic biallelic RFC1 repeat expansions","authors":"Calezis Claudia, Bonello‐Palot Nathalie, Verschueren Annie, Azulay Jean‐Philippe, Fortanier Etienne, Grapperon Aude‐Marie, Kouton Ludivine, Gallard Julien, Salort‐Campana Emmanuelle, Attarian Shahram, Delmont Emilien","doi":"10.1002/mus.28257","DOIUrl":"https://doi.org/10.1002/mus.28257","url":null,"abstract":"Introduction/AimsCerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS) is caused by RFC1 expansions. Sensory neuronopathy, polyneuropathy, and involvement of motor, autonomic, and cranial nerves have all been described with RFC1 expansions. We aimed to describe the electrodiagnostic features of patients with RFC1 expansions through multimodal electrophysiological investigations.MethodsThirty‐five patients, with a median age of 70 years, and pathologic biallelic repeat expansions in the RFC1 gene, were tested for motor and sensory nerve conduction, flexor carpi radialis (FCR) and soleus H‐reflexes, blink reflex, electrochemical skin conductance, sympathetic skin response (SSR), and heart rate variability with deep breathing (HRV).ResultsOnly 16 patients (46%) exhibited the full clinical CANVAS spectrum. Distal motor amplitudes were normal in 30 patients and reduced in the legs of five patients. Distal sensory amplitudes were bilaterally reduced in a non‐length dependent manner in 30 patients. Conduction velocities were normal. Soleus H‐reflexes were abnormal in 19/20 patients of whom seven had preserved Achilles reflexes. FCR H‐reflexes were absent or decreased in amplitude in 13/14 patients. Blink reflex was abnormal in 4/19 patients: R1 latencies for two patients and R2 latencies for two others. Fourteen out of 31 patients (45%) had abnormal results in at least one autonomic nervous system test, either for ESC (12/31), SSR (5/14), or HRV (6/19).DiscussionLess than half of the patients with RFC1 expansions exhibited the full clinical CANVAS spectrum, but nearly all exhibited typical sensory neuronopathy and abnormal H‐reflexes. Involvement of small nerve fibers and brainstem neurons was less common.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142247273","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

Evaluation and management of dyspnea as the dominant presenting feature in neuromuscular disorders 以呼吸困难为主要表现特征的神经肌肉疾病的评估和管理

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-13 DOI: 10.1002/mus.28243

Mansoureh Mamarabadi, Sarah Mauney, Yuebing Li, Loutfi S. Aboussouan

{"title":"Evaluation and management of dyspnea as the dominant presenting feature in neuromuscular disorders","authors":"Mansoureh Mamarabadi, Sarah Mauney, Yuebing Li, Loutfi S. Aboussouan","doi":"10.1002/mus.28243","DOIUrl":"https://doi.org/10.1002/mus.28243","url":null,"abstract":"Dyspnea is a common symptom in neuromuscular disorders and, although multifactorial, it is usually due to respiratory muscle involvement, associated musculoskeletal changes such as scoliosis or, in certain neuromuscular conditions, cardiomyopathy. Clinical history can elicit symptoms such as orthopnea, trepopnea, sleep disruption, dysphagia, weak cough, and difficulty with secretion clearance. The examination is essential to assist with the diagnosis of an underlying neurologic disorder and determine whether dyspnea is from a cardiac or pulmonary origin. Specific attention should be given to possible muscle loss, use of accessory muscles of breathing, difficulty with neck flexion/extension, presence of thoraco‐abdominal paradox, conversational dyspnea, cardiac examination, and should include a detailed neurological examination directed at the suspected differential diagnosis. Pulmonary function testing including sitting and supine spirometry, measures of inspiratory and expiratory muscle strength, cough peak flow, sniff nasal inspiratory pressure, pulse oximetry, transcutaneous CO2, and arterial blood gases will help determine the extent of the respiratory muscle involvement, assess for hypercapnic or hypoxemic respiratory failure, and qualify the patient for noninvasive ventilation when appropriate. Additional testing includes dynamic imaging with sniff fluoroscopy or diaphragm ultrasound, and diaphragm electromyography. Polysomnography is indicated for sleep related symptoms that are not otherwise explained. Noninvasive ventilation alleviates dyspnea and nocturnal symptoms, improves quality of life, and prolongs survival. Therapy targeted at neuromuscular disorders may help control the disease or favorably modify its course. For patients who have difficulty with secretion clearance, support of expiratory function with mechanical insufflation‐exsufflation, oscillatory devices can reduce the aspiration risk.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142247275","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

Assessing hepatitis B virus serologies when transitioning patients from intravenous immune globulin therapy to rituximab for the treatment of autoimmune neuromuscular diseases 在患者从静脉注射免疫球蛋白治疗过渡到利妥昔单抗治疗自身免疫性神经肌肉疾病时评估乙型肝炎病毒血清学情况

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-13 DOI: 10.1002/mus.28253

Nicole M. Hahn, Lara W. Katzin

{"title":"Assessing hepatitis B virus serologies when transitioning patients from intravenous immune globulin therapy to rituximab for the treatment of autoimmune neuromuscular diseases","authors":"Nicole M. Hahn, Lara W. Katzin","doi":"10.1002/mus.28253","DOIUrl":"https://doi.org/10.1002/mus.28253","url":null,"abstract":"Introduction/AimsIntravenous immune globulin (IVIG) has been used as early treatment for autoimmune neuromuscular diseases, but due to cost and frequency, may be switched to rituximab. Rituximab and other B‐cell‐depleting medications require screening of hepatitis B virus (HBV) serologies given the risk of HBV reactivation (HBVr). We aimed to describe the incidence and characteristics of passively transferred antiviral serologies from IVIG and how to differentiate between passive antibody transfer and resolved HBV infection.MethodsThis was a single‐center descriptive study of neurology patients prescribed rituximab and IVIG. Retrospective medical record reviews were performed and patient‐specific variables were collected.ResultsTwelve patients had reactive anti‐HBc results after starting IVIG, but only 9 were confirmed to have reactive anti‐HBc from passive transfer. Whether reactive anti‐HBc in the remaining three patients was from passive IVIG transfer could not be confirmed. Five patients with reactive anti‐HBc results during rituximab screening did not have pre‐IVIG anti‐HBc results for comparison and were started on antiviral prophylaxis. Reactive anti‐HBc serologies changed to nonreactive after IVIG discontinuation 44–321 days after the last IVIG infusion.DiscussionThis study confirms IVIG can passively transfer anti‐HBc serologies in a neurologic cohort. Ideally, HBV serologies would be checked before starting IVIG to help later determine if passive transfer occurred. With the increasing use of B‐cell‐depleting medications for neuromuscular conditions, it is important for providers to be knowledgeable on the interpretation of HBV serologies for patients on IVIG and to ensure implementation of an HBVr prophylaxis management strategy for patients when appropriate.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142247274","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

Hypogammaglobulinemia and infection risk in myotonic dystrophy type 1 低丙种球蛋白血症与 1 型肌营养不良症的感染风险

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-13 DOI: 10.1002/mus.28247

Shadi El‐Wahsh, Katrina Morris, Sandhya Limaye, Sean Riminton, Alastair Corbett, James D. Triplett

{"title":"Hypogammaglobulinemia and infection risk in myotonic dystrophy type 1","authors":"Shadi El‐Wahsh, Katrina Morris, Sandhya Limaye, Sean Riminton, Alastair Corbett, James D. Triplett","doi":"10.1002/mus.28247","DOIUrl":"https://doi.org/10.1002/mus.28247","url":null,"abstract":"Introduction/AimsHypogammaglobulinemia is a common yet under‐recognized feature of myotonic dystrophy type 1 (DM1). The aims of our study were to determine the frequency of immunoglobulin G (IgG) deficiency in our cohort, to examine the association between immunoglobulin levels and cytosine–thymine–guanine (CTG) repeat length in the DMPK gene, and to assess whether IgG levels are associated with an increased risk of infection in DM1 patients.MethodsWe conducted a single‐center, retrospective cross‐sectional study of 65 adult patients with DM1 who presented to the Neuromuscular Clinic at Concord Repatriation General Hospital, Sydney, Australia, between January 2002 and January 2022. We systematically collected and analyzed clinical, laboratory, and genetic data for all patients with available serum electrophoresis and/or IgG level results.ResultsForty‐one percent of DM1 patients had IgG deficiency despite normal lymphocyte counts, IgA, IgM, and albumin levels. There was an association between CTG repeat expansion size and the degree of IgG deficiency (F = 6.3, p = .02). There was no association between IgG deficiency and frequency of infection in this group (p = .428).DiscussionIgG deficiency is a frequent occurrence in DM1 patients and is associated with CTG repeat expansion size. Whether hypogammaglobulinemia is associated with increased infection risk in DM1 is unclear. A prospective multicenter cohort study is needed to evaluate this.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142247276","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

Magnetic resonance neurography in the diagnosis of neurological subtypes of thoracic outlet syndrome 磁共振神经成像在胸廓出口综合征神经亚型诊断中的应用

IF 3.4 3区医学

Muscle & Nerve Pub Date : 2024-09-10 DOI: 10.1002/mus.28246

Emily J. Davidson, Ek T. Tan, Darryl B. Sneag

{"title":"Magnetic resonance neurography in the diagnosis of neurological subtypes of thoracic outlet syndrome","authors":"Emily J. Davidson, Ek T. Tan, Darryl B. Sneag","doi":"10.1002/mus.28246","DOIUrl":"https://doi.org/10.1002/mus.28246","url":null,"abstract":"Neurological thoracic outlet syndrome (TOS) can be challenging to diagnose, particularly given its described subtypes of neurogenic TOS (NTOS) and disputed TOS (DTOS) that exhibit variable clinical presentations and etiologies. The diagnostic workup of TOS often includes magnetic resonance neurography (MRN) of the brachial plexus. Specific MRN imaging modifications for TOS evaluation are required to maximize spatial and contrast resolution to increase the conspicuity of nerve segments and their relationships to surrounding osseous structures. Dynamic assessment with arm positioning is used to evaluate outlet narrowing and compression of the plexus. Individual nerve segments are interrogated for their longitudinal and cross‐sectional morphologies and signal characteristics. In patients with NTOS, MRN may reveal focal impingement of the C8/T1 nerve roots and/or lower trunk with accompanying abnormal T2‐weighted signal hyperintensity. Predisposing anatomical entities include cervical ribs, rib synostoses, hypertrophic callous following clavicular fracture, remnant first thoracic rib from prior incomplete resection, and variable perineural scarring. In comparison, DTOS patients frequently demonstrate signal hyperintensity and enlargement of the mid plexus (trunk and division level), with narrowing of the costoclavicular interval. Following comprehensive diagnostic workup that frequently includes electrodiagnostic testing, patients are directed to different management pathways. Nonsurgical management is considered for all cases of DTOS; all patients with NTOS or DTOS who fail conservative treatment warrant referral for a surgical opinion. If surgery is pursued, MRN can be helpful in preoperative planning.","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142205743","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