Muscle & Nerve最新文献

{"title":"Associations Between End-Stage ALS Care and Specialty Palliative Care: A Hypothesis-Generating Study.","authors":"Christi M Lero, Annabelle Yang, Elyse Everett, Kyle A Pitzer, Kelly McCoy Gross, Karla T Washington","doi":"10.1002/mus.28370","DOIUrl":"10.1002/mus.28370","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Amyotrophic lateral sclerosis (ALS) care is typically delivered via a multidisciplinary approach that may include specialty palliative care (SPC). Opportunities for SPC to enhance ALS care have been identified; however, investigation of these proposed benefits is scant. In this exploratory study, investigators examined associations between receipt of SPC and variables particularly relevant to end-stage ALS.</p><p><strong>Methods: </strong>Researchers reviewed electronic health records for all patients with ALS who received standard ALS care from one Midwestern US academic medical center and died between January 1, 2020, and June 30, 2022 (N = 156). Receipt of SPC, duration of illness, hospice enrollment and length of service, report of a healthcare proxy, documentation of a healthcare proxy, participation in goals of care conversations, and location of death were examined.</p><p><strong>Results: </strong>Patients who received SPC (59%), had lower mean forced vital capacity (FVC) (p < 0.05), and more often used respiratory support (p < 0.001), participated in goals of care conversations (p < 0.001), reported a healthcare proxy (p < 0.01), and enrolled in hospice (p < 0.001) than patients who received standard care alone. No differences between groups were found in duration of illness (mean = 51.7 months), use of assistive feeding, Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) scores (mean = 32.1), documentation of a healthcare proxy, length of hospice stay (mean = 47.3 days), or location of death.</p><p><strong>Discussion: </strong>Clinical characteristics and end-of-life outcome differences between groups support further investigation of the proposed benefits of SPC regarding hospice enrollment, report of healthcare proxies, and documented goals of care conversations.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"632-638"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11938423/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399477","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}

{"title":"Frequency and Severity of Myasthenia Gravis Exacerbations Associated With the Use of Ciprofloxacin, Levofloxacin, and Azithromycin.","authors":"Sanem Pinar Uysal, Yadi Li, Nicolas R Thompson, Yuebing Li","doi":"10.1002/mus.28410","DOIUrl":"https://doi.org/10.1002/mus.28410","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The true frequency and severity of myasthenia gravis (MG) exacerbation associated with the usage of fluoroquinolone and macrolide antibiotics remain unknown. We aimed to investigate the association between ciprofloxacin, levofloxacin, azithromycin, and MG exacerbation.</p><p><strong>Methods: </strong>A retrospective review was performed on MG patients seen at a single institution between 2002 and 2022, who received ciprofloxacin, levofloxacin, or azithromycin. Amoxicillin usage was chosen for comparison. The strength of association between antibiotic usage and MG exacerbation was scored using the Adverse Drug Reactions Probability Scale. A mixed-effects logistic regression model was constructed to evaluate predictors of antibiotic-associated MG exacerbation (AAMGE).</p><p><strong>Results: </strong>365 patients had a total of 918 episodes of antibiotic usage (n = 339 for ciprofloxacin, n = 187 for levofloxacin, n = 392 for azithromycin). Frequencies of MG exacerbation following antibiotic use were: 8 (2.4%) for ciprofloxacin, 3 (1.6%) for levofloxacin, 6 (1.5%) for azithromycin, and 17 (1.9%) for all. Six patients had impending crisis/crisis, and 9 required rescue therapy. MG exacerbation was associated with MG-related hospitalization or ED visit in the preceding 6 months (p = 0.012), female sex (p = 0.023) and diabetes (p = 0.032). Infection was the most common confounder in exacerbations (88.2%). MG exacerbation was seen in 8/603 (1.3%) episodes of amoxicillin use, without a significant difference in frequencies of AAMGE among the four antibiotics (p = 0.68).</p><p><strong>Discussion: </strong>Usage of ciprofloxacin, levofloxacin, or azithromycin was associated with MG exacerbation in less than 2.5% of episodes of antibiotic use. Underlying infection may play a role in AAMGE. As AAMGE can be severe, decision-making regarding the use of these antibiotics should be individualized.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753411","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":"Electrophysiological Abnormalities in Finger Extension Weakness and DOwnbeat Nystagmus Motor Neuron Disease: Three New Patients and Review of the Literature.","authors":"Julian Theuriet, Emilien Bernard, Nathalie Guy, Frédéric Taithe, Cécilia Even, Thierry Maisonobe, Aude Sangaré, Pierre Lardeux, Caroline Froment Tilikete, Philippe Couratier, Timothée Lenglet, Antoine Pegat","doi":"10.1002/mus.28357","DOIUrl":"10.1002/mus.28357","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Finger Extension Weakness and DOwnbeat Nystagmus Motor Neuron Disease (FEWDON-MND) is characterized by motor weakness predominantly affecting finger extension, accompanied by downbeat nystagmus. To date, only 11 patients have been reported. The present study adds a further three and aims to provide a more detailed description of the electrodiagnostic features of these patients.</p><p><strong>Methods: </strong>We present the clinical and electrophysiological features of three French patients from specialized motor neuron centers and review the electrophysiological findings of previously reported patients.</p><p><strong>Results: </strong>These three patients presented with pure motor weakness affecting finger extension and downbeat nystagmus. They exhibited a slowly progressive disease course without respiratory involvement. Nerve conduction studies showed decreased compound muscle action potential amplitudes in the extensor indicis muscles. Abnormal spontaneous activity on needle electromyography (EMG) was rare in two patients, absent in one, and otherwise limited to weak muscles. Additionally, chronic motor axon loss features suggestive of motor neuronopathy were seen in our patients. Importantly, they were also detected in distant asymptomatic muscles.</p><p><strong>Discussion: </strong>The three patients reported here confirm the typical phenotype of FEWDON-MND, characterized by slowly progressive distal motor weakness initially affecting finger extension, associated with downbeat nystagmus. Although chronic motor axon loss features have been found in all reported patients, our three patients show that active denervation can be absent or rare. Thus, finger drop and diffuse chronic neurogenic changes on EMG should lead clinicians to look for downbeat nystagmus and to consider FEWDON-MND.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"644-650"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11887523/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143024181","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}

{"title":"Establishing Reference Values in the Neuromuscular Ultrasound Laboratory Using the e-Norms Method: A Feasibility Study.","authors":"Michał Błaż, Agnieszka Kułaga, Monika Ostrowska, Ewa Maludzińska, Michał Michalski, Joe F Jabre","doi":"10.1002/mus.28347","DOIUrl":"10.1002/mus.28347","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The standard procedure to establish reference values in a neuromuscular laboratory involves examining healthy controls, as nerve size varies with the population and muscle echo intensity (EI) is device-specific. We aimed to derive these reference values by extrapolation from a studied sample (the e-norms method), compare them with published reference values, and determine their diagnostic accuracy.</p><p><strong>Methods: </strong>We retrospectively analyzed data from consecutive patients who underwent nerve and/or muscle ultrasound in our ultrasound laboratory, which is a tertiary referral center for neuromuscular diseases in Southern Poland in the years 2018-2023. We plotted the cross-sectional area (CSA) of the nerve segments and EI of the individual muscles and derived descriptive statistics from the plateau of the e-norms curve. We compared the mean difference of the nerve size to other studies and determined the ability of the muscle EI to discriminate myopathic versus healthy subjects using receiver operator characteristics curves.</p><p><strong>Results: </strong>We analyzed 1124 nerves and 1154 muscles from 109 and 99 subjects, respectively. The derived reference values for nerve CSA were mostly concordant with other studies, including all nerve segments in the legs and with the exception of compression sites and the most proximal nerve segments in the arms. Using the reference values for muscle EI established with the e-norms method, we were able to discriminate myopathic from healthy subjects with 85% sensitivity and 92% specificity.</p><p><strong>Discussion: </strong>The e-norms is a feasible method to establish reference values in the neuromuscular ultrasound laboratory.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"543-551"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143008678","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":"Are Women Physicians Underrecognized for National Awards in Neuromuscular and Electrodiagnostic Medicine? An Observational Study.","authors":"Emma A Ali Bateman, Caitlin Cassidy, Rachel Reardon, Jamie L Fleet","doi":"10.1002/mus.28350","DOIUrl":"10.1002/mus.28350","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Institutions and organizations, including the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM), have committed to embracing principles of equity, diversity, and inclusion. Notwithstanding this commitment, studies repeatedly demonstrate that women physicians are less likely to receive awards in medicine and research compared to their male counterparts. Whether women physicians are less likely to be recognized with AANEM awards is unknown. The objective of this study was to evaluate whether there is a gender disparity in the AANEM's annual awards.</p><p><strong>Methods: </strong>In this retrospective observational study, lists of award winners were obtained from the AANEM website. Award winners' gender was assigned by three independent reviewers based on searches of public professional websites according to established methodology. Data were analyzed using descriptive statistics.</p><p><strong>Results: </strong>Of 154 physician awards from 1957 to 2023, 24 (15.6%) were awarded to women and 135 (84.4%) to men. The first woman to win an AANEM award was in 2003. As the number of award categories increased over time (from 1 pre-1994 to 9 as of 2019), so too did the proportion of women winners. From 1994 to 2003, 3.4% of AANEM awardees were women compared to 17.1% from 2004 to 2013 and 18% from 2014 to 2023. Even over time, the greatest disparities existed for the Distinguished Physician/Researcher and Lifetime Achievement awards.</p><p><strong>Discussion: </strong>For the AANEM, there is a notable gender gap in physician awards, but this gap has narrowed over time. Further efforts to address systemic barriers contributing to this disparity are warranted.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"639-643"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11887524/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143008655","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}

{"title":"Identifying Daytime Hypercapnia Using Transcutaneous Carbon Dioxide Monitoring in Patients With Amyotrophic Lateral Sclerosis.","authors":"Alexander H Morrison, Jose Victor Jimenez, Jesse Y Hsu, Lauren Elman, Philip J Choi, Jason Ackrivo","doi":"10.1002/mus.28366","DOIUrl":"10.1002/mus.28366","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Respiratory failure from hypoventilation is the most common cause of death in amyotrophic lateral sclerosis (ALS). However, ALS care rarely assesses hypercapnia, a physiologic measure of hypoventilation. We investigated the prevalence and clinical significance of daytime hypercapnia measured by transcutaneous carbon dioxide (tcCO₂) monitoring in patients with ALS.</p><p><strong>Methods: </strong>This retrospective study included patients seen at two ALS clinics in the United States between 2012 and 2024 who had tcCO₂ measured concurrently with pulmonary function tests (PFTs), which included forced vital capacity (FVC) and, at one site, maximum inspiratory pressure (MIP). We assessed the prevalence of hypercapnia (tcCO₂ > 45 mmHg), the sensitivity and specificity of patient symptoms and PFTs for hypercapnia, and the relationship between hypercapnia and survival.</p><p><strong>Results: </strong>Daytime hypercapnia was present in 33/328 (10%) patients at baseline. Hypercapnia was associated with an increased rate of death (aHR 2.1, 95% CI 1.4-3.3). Orthopnea or dyspnea was 70% sensitive for hypercapnia (95% CI 51%-84%). Absolute value of MIP (|MIP|) < 60 cmH₂O was 95% sensitive (95% CI 74%-100%) and 22% specific (95% CI 16%-30%), FVC < 50% predicted was 33% sensitive (95% CI 18%-52%) and 82% specific (95% CI 78%-87%), and FVC < 80% predicted was 85% sensitive (95% CI 68%-95%) and 31% specific (95% CI 26%-36%) for hypercapnia.</p><p><strong>Discussion: </strong>TcCO₂ monitoring identified strengths and weaknesses of PFTs in identifying hypercapnia in ALS. We found high sensitivity of |MIP| < 60 cmH₂O and FVC < 80% predicted and high specificity of FVC < 50% predicted. Prospective studies should investigate the optimal clinical role for tcCO₂.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"611-619"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11887530/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143399487","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}

{"title":"Corneal Confocal Microscopy Identifies Corneal Nerve Loss in Patients With Guillain-Barré Syndrome.","authors":"Adnan Khan, Mohammed Alhatou, Liaquat Ali, Gholam Adeli, Georgios Ponirakis, Hoda Gad, Abeer Sabry Safan, Rashaa K M Aldabbagh, Ioannis N Petropoulos, Ali Alsalahat, Rayaz A Malik","doi":"10.1002/mus.28408","DOIUrl":"https://doi.org/10.1002/mus.28408","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Patients with Guillain-Barré syndrome (GBS) commonly present with sensory loss and weakness, but they may also have neuropathic pain, elevated thermal thresholds, and intraepidermal nerve fiber loss. The primary aim of this study was to assess for evidence of small nerve fiber damage in patients with GBS.  METHODS: Nine patients with GBS and 15 age-matched healthy controls underwent CCM to quantify corneal nerve fiber density (CNFD), corneal nerve fiber length (CNFL), and corneal nerve branch density (CNBD), Douleur Neuropathique en 4 (DN4) to assess neuropathic pain, electrochemical skin conductance (ESC), vibration perception threshold (VPT), and nerve conduction studies.</p><p><strong>Results: </strong>CNFD (p = 0.008), CNFL (p = 0.011), CNBD (p = 0.005), and ESC on the hands (p = 0.024) and the feet (p = 0.046) were lower, whereas DN4 (p = 0.007) was higher in patients with GBS compared to healthy controls.</p><p><strong>Discussion: </strong>Patients with GBS have evidence of small nerve fiber damage as evidenced by corneal nerve fiber loss, neuropathic pain, and sudomotor dysfunction. CCM may add to the toolbox for the evaluation of small nerve fiber involvement in patients with GBS.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143753402","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":"Amyotrophic lateral sclerosis represents corticomotoneuronal system failure.","authors":"Andrew Eisen, Steve Vucic, Matthew C Kiernan","doi":"10.1002/mus.28290","DOIUrl":"10.1002/mus.28290","url":null,"abstract":"<p><p>Several decades have passed since the anterograde corticomotoneuronal hypothesis for amyotrophic lateral sclerosis (ALS) was proposed. The intervening years have witnessed its emergent support based on anatomical, pathological, physiological, neuroimaging, and molecular biological studies. The evolution of an extensive corticomotoneuronal system appears restricted to the human species, with ALS representing a uniquely human disease. While some, very select non-human primates have limited corticomotoneuronal projections, these tend to be absent in all other animals. From a general perspective, the early clinical features of ALS may be considered to reflect failure of the corticomotoneuronal system. The characteristic loss of skilled motor dexterity involving the limbs, and speech impairment through progressive bulbar dysfunction specifically involve those motor units having the strongest corticomotoneuronal projections. A similar explanation likely underlies the unique \"split phenotypes\" that have now been well characterized in ALS. Large Betz cells and other pyramidal corticomotoneuronal projecting neurons, with their extensive dendritic arborization, are particularly vulnerable to the elements of the ALS exposome such as aging, environmental stress and lifestyle changes. Progressive failure of the proteosome impairs nucleocytoplasmic shuffling and induces toxic but soluble TDP-43 to aggregate in corticomotoneurons. Betz cell failure is further accentuated through dysfunction of its profuse dendritic arborizations. Clarification of system specific genomes and neural networks will likely promote the initiation of precision medicine approaches directed to support the key structure that underlies the neurological manifestations of ALS, the corticomotoneuronal system.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"499-511"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11887532/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142605720","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}

{"title":"Incidence of Radiologically Confirmed Fractures in Adults With Duchenne Muscular Dystrophy.","authors":"Gavin Langlands, Jennifer McKechnie, Maria E Farrugia, Sze Choong Wong","doi":"10.1002/mus.28355","DOIUrl":"10.1002/mus.28355","url":null,"abstract":"<p><strong>Introduction/aims: </strong>An increased risk of low trauma fractures is well documented in children and adolescents with duchenne muscular dystrophy (DMD). There is limited evidence regarding the fracture incidence of adults with DMD. The aim of this study was to examine radiologically confirmed fractures in adults with DMD and review bone health monitoring.</p><p><strong>Methods: </strong>This was a retrospective review of all adult males ≥ 16 years with DMD under the care of adult physicians in the West of Scotland (2013-2022). Information regarding fractures, bone health monitoring, and bisphosphonate therapy was collected.</p><p><strong>Results: </strong>Thirty-six men (median age at first visit 18.8 years) with DMD were included. Twelve were taking corticosteroids at first review, and a further 12 had previously been taking corticosteroids that were discontinued in childhood or adolescence. The fracture incidence rate was higher in the corticosteroid group (888.9 per 10,000 person years (95% CI 242.2-2275.9)) than in those not on corticosteroids (156.3 per 10,000 person years (95% CI 32.2-456.6)). Eighteen had lateral spine radiographs for vertebral fracture assessment and 15 had a DXA scan for bone density assessment during the follow-up period.</p><p><strong>Discussion: </strong>The fracture incidence in adult men with DMD is more than double that of UK men aged 18-49 years old, with an even higher incidence in those treated with corticosteroids. Fewer than half of the study population underwent bone monitoring. There is a need for enhanced clinical guidance for the monitoring and management of osteoporosis during transition and throughout adulthood.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"558-563"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143024183","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":"Thoracic Electric Impedance Tomography Detects Lung Volume Changes in Amyotrophic Lateral Sclerosis.","authors":"Gregory Hansen, Adam Shaw, Kala Bolt, Ryan Verity, Richard T Nataraj, Kerri Lynn Schellenberg","doi":"10.1002/mus.28354","DOIUrl":"10.1002/mus.28354","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Spirometry is the conventional means to measure lung function in amyotrophic lateral sclerosis (ALS), but is dependent on patient effort and bulbar strength. We aimed to use electric impedance tomography (EIT), an emerging non-invasive imaging modality, to measure dynamic lung volume changes.</p><p><strong>Methods: </strong>Twenty-one patients with ALS underwent sitting and supine spirometry for forced vital capacity (FVC), and sitting and supine EIT. There were 13 patients in the high FVC group (FVC ≥ 80% predicted) and 8 in the low FVC group (FVC < 80% predicted). Additional demographic and clinical data were collected from clinical records.</p><p><strong>Results: </strong>Only the low FVC group had significant loss of lung volumes in the supine position (R ² = 0.89 and p < 0.001). The supine volume loss measurement at 10 min correlated with sitting (r ² = 0.47) and supine FVC (r ² = 0.36), maximum inspiratory (r ² = -0.44) and expiratory pressures (r ² = 0.36) (MIP and MEP), and the ALS Functional Rating Scale-Revised (ALSFRS-R) dyspnea subscore (r ² = 0.36).</p><p><strong>Discussion: </strong>EIT is an emerging alternative to existing measures of lung function in ALS, but without need for patient effort or bulbar strength. Significant losses in lung volume are seen on supine compared to upright position in patients with respiratory dysfunction. Further study is needed to determine relationships to existing clinical measures.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"552-557"},"PeriodicalIF":2.8,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11887520/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143033742","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}