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Predictive Value of Perioperative Blood Lactic Acid Levels for Postoperative Crisis in Myasthenia Gravis Patients Undergoing Thymectomy.
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-14 DOI: 10.1002/mus.28342
Jianyong Zou, Xiaojing Yao, Zhihao Liu, Zhenguo Liu, Haoshuai Zhu, Xin Zhang, Zhenguang Chen, Chunhua Su
{"title":"Predictive Value of Perioperative Blood Lactic Acid Levels for Postoperative Crisis in Myasthenia Gravis Patients Undergoing Thymectomy.","authors":"Jianyong Zou, Xiaojing Yao, Zhihao Liu, Zhenguo Liu, Haoshuai Zhu, Xin Zhang, Zhenguang Chen, Chunhua Su","doi":"10.1002/mus.28342","DOIUrl":"https://doi.org/10.1002/mus.28342","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Postoperative myasthenic crisis (POMC), which occurs specifically after thymectomy in myasthenia gravis (MG) patients, is a serious complication with known risk factors such as prior myasthenic crisis. However, the predictive value of perioperative blood lactic acid levels (BLAL) for POMC remains unclear. This study aims to determine whether changes in perioperative BLAL can predict POMC in MG patients undergoing thymectomy.</p><p><strong>Methods: </strong>A total of 340 patients diagnosed with MG and undergoing thymectomy at the First Affiliated Hospital of Sun Yat-sen University were enrolled (January 2008-September 2018). Multivariate logistic regression analyses were employed to discern independent factors linked with POMC.</p><p><strong>Results: </strong>Among the patients with POMC, notable differences including higher Myasthenia Gravis Foundation of America (MGFA) stage, and history of preoperative myasthenic crisis were observed. Higher postoperative lactic acid levels and the extent of changes were more prevalent in the POMC group. The multivariate analysis unveiled history of myasthenic crisis (odds ratio, OR: 67.18), postoperative BLAL change ratio greater than 50% (OR: 2.86), the video-assisted thoracoscopic surgery (VATS) approach (OR: 4.33), and higher preoperative BLAL (OR per unit: 2.68) were associated with POMC. Both continuous and grouped lactic acid models demonstrated a good predictive capability, yielding area under the curve (AUC) values of 0.84 and 0.83, respectively. The optimal threshold for 24-h postoperative BLAL was 1.98 mmol/L.</p><p><strong>Discussion: </strong>These findings offer valuable insights for clinical decision-making and monitoring of prognosis in managing patients with MG. Future research should explore further the underlying mechanisms linking elevated lactate levels to POMC.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143414622","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
Longitudinal Outcomes Among Patients With Duchenne Muscular Dystrophy: A Canadian Retrospective Population-Based Study.
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-13 DOI: 10.1002/mus.28368
Christina Qian, Alexa C Klimchak, Shelagh M Szabo, Katherine L Gooch, Roxana Dragan, Heather J Prior, Jean K Mah
{"title":"Longitudinal Outcomes Among Patients With Duchenne Muscular Dystrophy: A Canadian Retrospective Population-Based Study.","authors":"Christina Qian, Alexa C Klimchak, Shelagh M Szabo, Katherine L Gooch, Roxana Dragan, Heather J Prior, Jean K Mah","doi":"10.1002/mus.28368","DOIUrl":"https://doi.org/10.1002/mus.28368","url":null,"abstract":"<p><strong>Aims: </strong>There are few long-term studies evaluating clinical outcomes and mortality among individuals with Duchenne muscular dystrophy (DMD); particularly using longitudinal health administrative claims data, reflecting populations managed in typical clinical practice. This study aimed to characterize DMD outcomes via a population-based database.</p><p><strong>Methods: </strong>Patients with DMD, diagnosed between 01/1979 and 03/2020 at ≤ 10 years of age, were identified using the Manitoba Population Research Data Repository housed at the Manitoba Centre for Health Policy. De-identified longitudinal administrative data from 1998 to 2020 were used to retrospectively assess frequencies and age at first observation of key DMD outcomes including scoliosis, cardiovascular-related complications, severe respiratory-related morbidities, and mortality. Survival analyses using Kaplan-Meier curves were used to describe attrition and estimate probability of patients remaining observation-free by age.</p><p><strong>Results: </strong>This study included 198 patients with median (IQR) follow-up of 9.6 (6.6-15.5) years. Corticosteroid use was observed in 26%, with a mean (SD) percentage of days covered of 31% (39%) from initiation to end of follow-up. Scoliosis observations were captured in 18% (median[IQR] age 12 [11-15] years at first observation), severe respiratory-related morbidities in 20% (14[6.5-18] years), and cardiovascular-related complications in 32% of the cohort (12.5[2-20.5] years). Mortality was observed in 14% of the cohort. Kaplan-Meier curves estimated 15% mortality by age 20 years and 20% by 25 years.</p><p><strong>Discussion: </strong>In a population-based data set with decades of follow-up, these data provide longitudinal observations of the substantial burden of DMD, and insight into contemporary estimates of mortality and treatment patterns in Canada.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143409259","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
Electrodiagnostic Assessment of Peri-Procedural Iatrogenic Peripheral Nerve Injuries and Rehabilitation.
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-12 DOI: 10.1002/mus.28364
Geoffrey K Seidel, Amber R Vocelle, Ian S Ackers, Kenneth A Scott, Curtis A Carl, Barent A G Bradt, Daniel Dumitru, Michael T Andary
{"title":"Electrodiagnostic Assessment of Peri-Procedural Iatrogenic Peripheral Nerve Injuries and Rehabilitation.","authors":"Geoffrey K Seidel, Amber R Vocelle, Ian S Ackers, Kenneth A Scott, Curtis A Carl, Barent A G Bradt, Daniel Dumitru, Michael T Andary","doi":"10.1002/mus.28364","DOIUrl":"https://doi.org/10.1002/mus.28364","url":null,"abstract":"<p><p>Iatrogenic nerve injuries are a significant concern for medical professionals and the patients affected. Peri-procedural nerve injuries result in functional deficits associated with pain and disability. The exact pathophysiology and etiology of peri-procedural nerve injuries are complex and often elude providers. The rates of injury to specific nerves are unclear and relate to both procedural and patient specific risk factors. Initial classification of the nerve injury into neurapraxia, axonotmesis, mixed nerve injury, or possible complete transection (neurotmesis) guides rehabilitation and management. Electrodiagnostic medical consultation at least four weeks post-injury, supplemented with nerve imaging (ultrasound and magnetic resonance imaging), can allow for accurate nerve injury classification. Supplemented with nerve imaging and detailed clinical evaluation, treatment, recovery and rehabilitation can be maximized. Recognizing nerves at risk associated with medical and surgical procedures can facilitate injury avoidance and early diagnosis. If a nerve injury is incomplete, in an optimized physiologic milieu (good glucose control, smoking cessation, etc.), there is a good potential for spontaneous (total or partial) improvement over time. Surgical referral should be considered for severe nerve injuries within 6 months, especially if there is concern for neurotmesis, and/or deteriorating nerve function. This review gives guidance for approaching peri-procedural peripheral nerve injuries, including the timing and the role of electrodiagnostic medical consultation including serial electrodiagnostic studies in management and rehabilitation.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399479","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
Amyotrophic Lateral Sclerosis, the Endocannabinoid System, and Exogenous Cannabinoids: Current State and Clinical Implications.
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-12 DOI: 10.1002/mus.28359
Travis T Denton, Gregory T Carter, Megan Goddard, Jeremy Weiss, Douglas L Weeks, Patrick Weydt, Ethan B Russo, Michael D Weiss
{"title":"Amyotrophic Lateral Sclerosis, the Endocannabinoid System, and Exogenous Cannabinoids: Current State and Clinical Implications.","authors":"Travis T Denton, Gregory T Carter, Megan Goddard, Jeremy Weiss, Douglas L Weeks, Patrick Weydt, Ethan B Russo, Michael D Weiss","doi":"10.1002/mus.28359","DOIUrl":"https://doi.org/10.1002/mus.28359","url":null,"abstract":"<p><p>A unifying mechanistic cause for amyotrophic lateral sclerosis (ALS) remains uncertain. Multiple pathophysiological processes appear to occur simultaneously. Cannabinoids, including delta-9-tetrahydrocannabinol (THC), cannabidiol (CBD), cannabigerol (CBG), and others found in cannabis, and cannabis extracts (CEs), appear to have activity in these pathogenic pathways, which have led to increasing interest in cannabinoids as therapeutic agents for ALS. The use of cannabinoids as a treatment strategy is substantiated by preclinical evidence suggesting a role for the endocannabinoid system (ECS) in ALS and other neurodegenerative disorders. Preclinical data indicate that cannabis and CEs have powerful antioxidative, anti-inflammatory, and neuroprotective effects in the SOD1<sup>G93A</sup> mouse model of ALS. The use of CEs in SOD1<sup>G93A</sup> murine models has been shown to prolong neuronal cell survival, which leads to delayed onset of the disease state, and slows progression of the disease. Although research in humans remains limited, a few studies suggest that cannabis and CBD, in humans, provide benefits for both motor symptoms, including rigidity, cramps, and fasciculations, and non-motor symptoms including sleep quality, pain, emotional state, quality of life, and depression. There remains a need for further, well-designed clinical trials to validate further the use of an individual cannabinoid, or a combination of cannabinoids, as a disease-modifying therapy for ALS.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399475","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
Surgical Strategies for Functional Upper Extremity Reconstruction After Spinal Cord Injury.
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-12 DOI: 10.1002/mus.28351
Christopher S Crowe, Yusha Katie Liu, Catherine M Curtin, Vincent R Hentz, Scott H Kozin, Ida K Fox, Michael J Berger
{"title":"Surgical Strategies for Functional Upper Extremity Reconstruction After Spinal Cord Injury.","authors":"Christopher S Crowe, Yusha Katie Liu, Catherine M Curtin, Vincent R Hentz, Scott H Kozin, Ida K Fox, Michael J Berger","doi":"10.1002/mus.28351","DOIUrl":"https://doi.org/10.1002/mus.28351","url":null,"abstract":"<p><p>Spinal cord injuries (SCI) can substantially affect independence and quality of life, particularly by limiting upper extremity function. Surgical reconstruction offers the potential to restore motion in the hand, wrist, and elbow for those with deficits following cervical spinal cord injury. Techniques such as tendon transfer, tenodesis, and arthrodesis-often used in combination-are well-established strategies for enhancing upper extremity function. Nerve transfers have more recently been employed and differ from other procedures in that they are often time sensitive and should be performed before permanent muscle atrophy occurs. A comprehensive preoperative evaluation, including clinical examination and electrodiagnostic assessment, is essential to determine the availability and strength of donor tendons and nerves. The International Classification of Surgery for the Hand in Tetraplegia (ICSHT) system is the most utilized surgical classification for determining muscle that can be used for reconstruction. Based on this classification, prioritization is given to restoring elbow extension, wrist extension, pinch, and grasp. Postoperative rehabilitative therapy balances the need for immobilization while preventing joint stiffness and may also incorporate cortical retraining strategies to activate tendon and nerve transfers. Ultimately, a collaborative, interdisciplinary approach is essential for assessing the injury, determining operative candidacy, selecting the optimal treatment strategy, and providing tailored rehabilitation. This article explores the classification of SCI as it pertains to the upper limb, provides an overview of surgical options, describes the preoperative clinical and electrodiagnostic evaluation process, and discusses reconstructive strategies aimed at improving functional outcomes in individuals with SCI.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399555","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
Musculoskeletal mimics for lumbosacral radiculopathy. Part 1: Theoretical considerations. 腰骶神经根病的肌肉骨骼模拟治疗。第一部分:理论考虑。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-01 Epub Date: 2024-11-05 DOI: 10.1002/mus.28280
Anthony E Chiodo, Shawn P Jorgensen
{"title":"Musculoskeletal mimics for lumbosacral radiculopathy. Part 1: Theoretical considerations.","authors":"Anthony E Chiodo, Shawn P Jorgensen","doi":"10.1002/mus.28280","DOIUrl":"10.1002/mus.28280","url":null,"abstract":"<p><p>The diagnosis of lumbosacral radiculopathy includes the exclusion of common musculoskeletal conditions that can cause similar symptoms. Neurology and physiatry physicians use history taking and physical examination findings to develop a differential diagnosis. Appropriate diagnostic testing is then utilized to narrow down this differential diagnosis to determine a working hypothesis of the cause of a patient's symptoms, leading to a treatment plan. There are stark limitations of patient symptoms and physical examination findings in making the diagnosis of lumbosacral radiculopathy and added value of a combination of symptoms and signs to distinguish patients with lumbosacral radiculopathy from patients with mimic disorders. Diagnostic tests have variable strengths and limitations in helping to confirm this diagnosis, contrasting the high sensitivity and lower specificity of magnetic resonance imaging (MRI) with the high specificity but lower sensitivity of electromyography (EMG). Further complexity is added to the task of making a diagnosis and setting a treatment plan by the fact that these disorders are common and interact with each other; they are present concomitantly in up to 25% of patients presenting for electrodiagnostic evaluation. A companion paper will review common musculoskeletal mimics of lumbosacral radiculopathy and provide tools to anchor testing for those conditions to the traditional neurological evaluation of lumbosacral radiculopathy.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"147-152"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142583831","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 BELS questionnaire: A novel screening tool for neurodevelopmental and psychiatric symptoms in pediatric dystrophinopathy. BELS 问卷:小儿肌营养不良症神经发育和精神症状的新型筛查工具。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-01 Epub Date: 2024-11-21 DOI: 10.1002/mus.28296
Natalie Truba, Seth Sorensen, Rachel Bearden, Brett Haley, Beverly Spray, Kathi Kinnett, Rachel Schrader, Aravindhan Veerapandiyan, Mary K Colvin
{"title":"The BELS questionnaire: A novel screening tool for neurodevelopmental and psychiatric symptoms in pediatric dystrophinopathy.","authors":"Natalie Truba, Seth Sorensen, Rachel Bearden, Brett Haley, Beverly Spray, Kathi Kinnett, Rachel Schrader, Aravindhan Veerapandiyan, Mary K Colvin","doi":"10.1002/mus.28296","DOIUrl":"10.1002/mus.28296","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Pediatric patients with dystrophinopathies [Becker and Duchenne muscular dystrophy (BDMD)] are more likely to have neurodevelopmental and neuropsychiatric conditions. This prospective pilot study tested a novel screening questionnaire developed to identify the common behavioral (B), emotional (E), learning (L), and social (S) difficulties in BDMD.</p><p><strong>Methods: </strong>A total of 45 caregivers of BDMD patients (ages 4-19 years) seen at the Arkansas Children's Hospital Dystrophinopathy Clinic completed the BELS questionnaire during standard clinic visits. To establish external validity, caregivers also completed four well-validated standardized questionnaires assessing overlapping symptoms [Pediatric Symptom Checklist-17 (PSC-17), Colorado Learning Difficulties Questionnaire (CLDQ), the obsessive compulsive disorder (OCD) subscale from the Revised Children's Anxiety and Depression Scale (RCADS), and Barkley Deficits in Executive Functioning Scale (BDEFS)].</p><p><strong>Results: </strong>Caregivers reported high rates of behavioral (>60%), emotional (50%-70%), learning (50%-70%), and social (30%-50%) concerns, even though relatively few patients had clinical diagnoses related to these symptoms. Passive suicidality (i.e., thoughts of death) were also common (22.2%; 10/45 patients). The BELS questionnaire was moderately to highly correlated with total scores on the four well-validated questionnaires (p < .001), and BELS subscales showed specificity. A BELS score of 21 corresponded with the established PSC-17 cutoff.</p><p><strong>Discussion: </strong>The BELS questionnaire shows promise as a screening questionnaire to identify neurobehavioral and neuropsychiatric symptoms common in dystrophinopathy. While BELS cannot currently be used as a standalone measure to make clinical diagnoses, identification of symptoms that warrant further follow-up may improve clinical care and quality of life.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"153-158"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11710981/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142682104","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
An Artifact in Concentric Needle EMG Recordings. 同心针肌电图记录中的伪影
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-01 Epub Date: 2024-12-12 DOI: 10.1002/mus.28312
Sanjeev D Nandedkar, Paul E Barkhaus
{"title":"An Artifact in Concentric Needle EMG Recordings.","authors":"Sanjeev D Nandedkar, Paul E Barkhaus","doi":"10.1002/mus.28312","DOIUrl":"10.1002/mus.28312","url":null,"abstract":"<p><strong>Introduction/aims: </strong>In healthy subjects, we observed high amplitude motor unit potential (MUP) waveforms that resembled the cannula potential (CP) with a positive sharp wave (PSW)-like waveform. We analyzed the source of this signal, its prevalence, and its effects on the analysis of electromyographic waveforms.</p><p><strong>Methods: </strong>Three channel recordings were performed to explore the contribution of the needle core and cannula to the MUP. In 7 control subjects with no neuromuscular disease, the EMG interference pattern was quantified for the amplitude asymmetry (AASM) of its positive and negative going peaks.</p><p><strong>Results: </strong>The large amplitude MUP resembling a CP with a PSW-type waveform is recorded by the needle core, not the cannula. With a slight change in needle position, its amplitude decreased and the waveform had a normal appearance. Thus, it is an artifact. Such potentials (AASM > 150%) are commonly encountered (frequency 7%) and occur in all muscles and subjects in this study.</p><p><strong>Discussion: </strong>The artifact is an enigma and appears related to the interaction between the needle and muscle fiber(s) similar to that in PSW signals. Failure to recognize these waveforms may result in a false impression of abnormality. AASM calculations can help confirm this artifact in automated analysis algorithms to preclude false positive neurogenic patterns.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"216-222"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142818743","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
X-Ray Fluoroscopy-Based Kinematic Analysis of Quadrupedal Locomotion in Slow and Fast Fatigue-Resistant Motor Neuron-Deleted Mice. 基于x射线透视的慢速和快速抗疲劳运动神经元缺失小鼠四足运动运动学分析。
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-01 Epub Date: 2024-12-18 DOI: 10.1002/mus.28324
Ayumu Ono, Daijiro Inomata, Lisa Ohgaki, Tenkei Koyama, Akiteru Maeno, Hidemi Misawa, Naomichi Ogihara
{"title":"X-Ray Fluoroscopy-Based Kinematic Analysis of Quadrupedal Locomotion in Slow and Fast Fatigue-Resistant Motor Neuron-Deleted Mice.","authors":"Ayumu Ono, Daijiro Inomata, Lisa Ohgaki, Tenkei Koyama, Akiteru Maeno, Hidemi Misawa, Naomichi Ogihara","doi":"10.1002/mus.28324","DOIUrl":"10.1002/mus.28324","url":null,"abstract":"<p><strong>Introduction/aims: </strong>VAChT-Cre is a transgenic mouse line targeting slow-twitch fatigue-resistant and fast-twitch fatigue-resistant motor neurons that innervate oxidative type I and type IIa muscle fibers. To ablate these neurons, VAChT-Cre mice were crossbred with NSE-DTA mice, leading to the expression of diphtheria toxin A after Cre-mediated excision. The resulting VAChT-Cre;NSE-DTA mice exhibited motor deficits, abnormal locomotion, muscular atrophy, and tremor, making them a useful model for studying motor neuron physiology and pathology. In this study, we conducted a kinematic analysis to examine their abnormal locomotor phenotype.</p><p><strong>Methods: </strong>The quadrupedal walking of VAChT-Cre;NSE-DTA and control mice along a 500 mm acrylic tunnel was analyzed using an X-ray fluoroscopic system. Stride duration, stride length, footfall patterns, and limb and trunk kinematics were quantified and compared between the two groups.</p><p><strong>Results: </strong>Our results demonstrated that VAChT-Cre;NSE-DTA mice walked more slowly than control mice (99.2 ± 43.5 mm/s vs. 120.5 ± 27.0 mm/s) and had a longer cycle duration (0.54 ± 0.19 s vs. 0.41 ± 0.09 s). In addition, the hindlimb was comparatively more flexed during the stance phase, the trunk was more rounded and humpbacked, and the cervix was lower in VAChT-Cre;NSE-DTA mice than in the control mice during locomotion.</p><p><strong>Discussion: </strong>These characteristic differences in the gait kinematics might be attributed to a malfunctioning of the motor units with slow-twitch fatigue-resistant and fast-twitch fatigue-resistant types in VAChT-Cre;NSE-DTA mice. The basic description of the locomotor characteristics of this transgenic mouse line may serve as a basis for future comparative analyses.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"257-264"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11708449/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142847114","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 Economic Burden of Myasthenia Gravis (MG): A Survey of Affected People and Their Families. 重症肌无力(MG)患者及其家庭的经济负担调查
IF 2.8 3区 医学
Muscle & Nerve Pub Date : 2025-02-01 Epub Date: 2024-12-18 DOI: 10.1002/mus.28313
Kathleen Yoder, Rachael Burris, Sujatha Gurunathan, Glenn Phillips, Kathy Perez, Ali A Habib, Pushpa Narayanaswami
{"title":"The Economic Burden of Myasthenia Gravis (MG): A Survey of Affected People and Their Families.","authors":"Kathleen Yoder, Rachael Burris, Sujatha Gurunathan, Glenn Phillips, Kathy Perez, Ali A Habib, Pushpa Narayanaswami","doi":"10.1002/mus.28313","DOIUrl":"10.1002/mus.28313","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Claims-based analyses have demonstrated high medical costs associated with myasthenia gravis (MG). We examined the economic burden of MG from the perspective of affected people and their families.</p><p><strong>Methods: </strong>The Muscular Dystrophy Association developed and conducted an online survey of people with MG and their caregivers between October 26 and December 6, 2021. Major themes were: MG-associated financial decisions, quality of life, employment and direct/indirect costs of diagnosis, healthcare insurance, medical visits, medications/treatments, medical equipment/devices, and caregiving.</p><p><strong>Results: </strong>Eight hundred people with MG and 238 caregivers responding on behalf of a person with MG completed the survey. Most respondents were white (85%) and about half (55%) had received a 4 year college degree or higher education. Respondents indicated that MG impacted their health, recreation, and finances. 70% reported making financial adjustments or seeking financial assistance. Annual out-of-pocket costs (mean, $15,798; median, $7750) were primarily driven by medications, cost to receive a diagnosis, and healthcare insurance premiums. People receiving infusions paid significantly more across all care domains. Demographic disparities were observed, with people of color, women, and those on disability paying significantly more to receive a diagnosis than their counterparts.</p><p><strong>Discussion: </strong>The economic burden on people with MG and their families is substantial, primarily driven by direct costs. There is a need to engage more broadly with the MG community for generalizable information. Respondents were self-selected, which may impact results. The results will be used to educate the public and inform advocacy work, with the goal of improving the lives of people with MG.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"244-251"},"PeriodicalIF":2.8,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142854957","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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