Muscle & NervePub Date : 2025-03-01Epub Date: 2024-12-29DOI: 10.1002/mus.28322
Christopher D Graham, Michael Rose, Victoria Edwards, Chiara Vari, Nicola O'Connell, Emma Taylor, Lance M McCracken, Aleksander Radunovic, Wojtek Rakowicz, Sam Norton, Trudie Chalder
{"title":"Long-Term Outcomes From a Randomized Controlled Trial of Acceptance and Commitment Therapy (ACT) Compared to Standard Medical Care for Improving Quality of Life in Muscle Disorders.","authors":"Christopher D Graham, Michael Rose, Victoria Edwards, Chiara Vari, Nicola O'Connell, Emma Taylor, Lance M McCracken, Aleksander Radunovic, Wojtek Rakowicz, Sam Norton, Trudie Chalder","doi":"10.1002/mus.28322","DOIUrl":"10.1002/mus.28322","url":null,"abstract":"<p><strong>Introduction/aims: </strong>A previous randomized controlled trial showed that guided self-help acceptance and commitment therapy plus standard medical care (ACT+SMC) was superior to standard medical care alone (SMC) for improving quality of life (QoL) and mood at 9-weeks post randomization in a sample of people with muscle disorders (MD). This follow-up study evaluated whether these effects were maintained in the longer term alongside individual patterns of response.</p><p><strong>Methods: </strong>The original study was a two-arm parallel group randomized controlled trial, which compared ACT+SMC to SMC. The primary outcome of QoL was assessed with the Individualized Neuromuscular Quality of Life Questionnaire. We recruited people with different MDs from UK National Health Service clinics and patient registries. In this follow-up study, we re-administered all outcome measures to participants at 6 months post randomization.</p><p><strong>Results: </strong>Questionnaires were completed by 109 participants (70.3% of the original sample). At six months, the adjusted group difference in QoL continued to favor ACT+SMC, which was significant with moderate effect size. Improvements in secondary outcomes of mood and aspects of psychological flexibility also favored ACT+SMC. Reliable improvement was evident in 33.9% of the ACT+SMC group and 5.7% of the SMC group. Reliable deterioration was uncommon following ACT+SMC (1.8% of participants.) DISCUSSION: The beneficial impacts of guided self-help ACT for QoL and mood were maintained in the longer-term. A third of participants showed response to this brief intervention, and negative individual outcomes were very rare. As is common in psychological interventions, there was a considerable group of non-responders.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"398-405"},"PeriodicalIF":2.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11799407/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142907284","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":"Pitfalls of the E-Ref Procedure: Tie Values and the Proportion of the Abnormal Data.","authors":"Keisuke Tachiyama, Takamichi Kanbayashi, Akiko Kawabata, Satoshi Hoshino, Yosuke Miyaji, Shunsuke Kobayashi, Hirofumi Maruyama, Masahiro Sonoo","doi":"10.1002/mus.28338","DOIUrl":"10.1002/mus.28338","url":null,"abstract":"<p><strong>Introduction: </strong>Extrapolated reference values (E-Ref) procedure is a new method for determining the cutoff value without collecting the control data. We tried to apply this method to determine the cutoff value for the distal motor latency of the median nerve (median DML). During this process, we found two pitfalls of the E-Ref method. First, the E-Ref procedure did not correctly work when the DML values measured with 0.1 ms accuracy frequently took on tie values. Second, the result was influenced by the proportion of abnormal values. This study investigated these issues.</p><p><strong>Methods: </strong>Data of the median DML were extracted from our laboratory database. To solve the problem of tie values, we tried a wider post-smoothing window in the original E-Ref method. We also devised a modified method conducting pre-smoothing. To see the effect of the proportion of abnormal data, we simulated many datasets having different proportion of abnormal data.</p><p><strong>Results: </strong>In total, 1016 DML values were identified. False deflections due to tie values were often identified as the E-Ref point using the original methods even using a wider window, resulting in unrealistically low values. Modified method was free from this drawback. For all methods, the E-Ref value increased as the proportion of abnormal values increased.</p><p><strong>Discussion: </strong>The problem of tie values, a pitfall of the E-Ref method, might be solved by pre-smoothing the data. The E-Ref value is influenced by the proportion of the normal data, and datasets containing less than 20% abnormal data may achieve appropriate results.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"435-441"},"PeriodicalIF":2.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142932313","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}
Muscle & NervePub Date : 2025-03-01Epub Date: 2025-01-03DOI: 10.1002/mus.28328
Corey Bacher, Pushpa Narayanswami, Mark Bromberg, Frank Buttgereit, Dubravka Dodig, Eoin P Flanagan, Chloe Gottlieb, John K Marshall, Rozalina G McCoy, Sara Mitchell, Pritish K Tosh, Partha S Sinha, Ruple S Laughlin, Charles D Kassardjian
{"title":"International Consensus Guidance for the Management of Glucocorticoid Related Complications in Neuromuscular Disease.","authors":"Corey Bacher, Pushpa Narayanswami, Mark Bromberg, Frank Buttgereit, Dubravka Dodig, Eoin P Flanagan, Chloe Gottlieb, John K Marshall, Rozalina G McCoy, Sara Mitchell, Pritish K Tosh, Partha S Sinha, Ruple S Laughlin, Charles D Kassardjian","doi":"10.1002/mus.28328","DOIUrl":"10.1002/mus.28328","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Glucocorticoid (GC)-related adverse reactions and risks are commonly seen during the treatment of immune-mediated and inflammatory neuromuscular disorders. There is wide variation in the management of associated complications. The aim of this study is to develop international consensus guidance on the management of GC-related complications in neuromuscular disorders.</p><p><strong>Methods: </strong>Through the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM), an international task force of 15 experts was convened to develop clinical guidance for the management of GC-related complications in neuromuscular patients. The RAND/UCLA appropriateness method (RAM) was used to develop consensus guidance statements. Initial guidance statements were crafted after a thorough literature review and were modified after anonymous panel input, with up to three rounds of voting via email to achieve consensus.</p><p><strong>Results: </strong>Statements were developed and achieved consensus for general care, monitoring of patients while on GC, osteoporosis prevention, vaccinations, infection screening, and Pneumocystis jiroveci pneumonia prophylaxis. A multidisciplinary approach to the management of GC-related complications was emphasized.</p><p><strong>Discussion: </strong>These formal consensus statements provide guidance to clinicians who use GC in the treatment of neuromuscular diseases regarding prevention and management of the more common associated adverse events and risks that arise with long and short-term GC use and serve as a springboard for investigation and updates.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"309-316"},"PeriodicalIF":2.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142922213","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}
Muscle & NervePub Date : 2025-03-01Epub Date: 2025-01-03DOI: 10.1002/mus.28332
Antony Winkel, Lauren Sanders, Mark Cook, Leslie Roberts
{"title":"High-Speed Video Blink Analysis Improves Detection of Facial Palsy in Early Guillain-Barré Syndrome.","authors":"Antony Winkel, Lauren Sanders, Mark Cook, Leslie Roberts","doi":"10.1002/mus.28332","DOIUrl":"10.1002/mus.28332","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Electrophysiological investigations in early Guillain-Barré Syndrome (GBS) can be nondiagnostic. Improved testing for facial weakness in the early phase of GBS may improve diagnostic processes, as such weakness is found in approximately 50% of patients with GBS. This work pilots the utility of high-speed video analysis to complement blink reflex testing in early GBS.</p><p><strong>Methods: </strong>This work prospectively evaluated consecutive patients admitted to a metropolitan teaching hospital in Melbourne, Australia, with suspected acute GBS within the first 14 days of neurological symptoms and compared them to a cohort of healthy controls. Blink reflex testing, mechanically-activated masseter reflexes, and analysis of high-speed video recordings of the evoked blinks were performed at admission (day 0), day 7, and day 21 (±2 days).</p><p><strong>Results: </strong>19 suspected GBS patients (12 GBS and 7 mimics) were compared to 22 healthy controls. At the first test, 83% of GBS patients and 29% of mimics demonstrated blink reflex abnormalities. 50% of GBS manifested video abnormalities (14% mimics), but abnormalities preceded electrophysiological changes in two GBS patients. The calculated reference values for peak lid velocity and lid excursion by video analysis were ~160 mm/s and 7-8 mm, respectively, with slightly different values for ipsilateral versus contralateral responses.</p><p><strong>Discussion: </strong>Combining high-speed video analysis and blink reflex testing improves the detection of facial involvement in early GBS and helps discriminate from mimic disorders. Further work in a larger cohort is required to validate the sensitivity and specificity of this technique.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"429-434"},"PeriodicalIF":2.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142922201","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}
Muscle & NervePub Date : 2025-03-01Epub Date: 2025-01-02DOI: 10.1002/mus.28329
Can Ebru Bekircan-Kurt, Sharmada Subramanian, Shannon Chagat, Samuel J Mackenzie, Megan Iammarino, Natalie Reash, Carson Richardson, Chang-Yong Tsao, Garey Noritz, Courtney Gushue, Kavitha Kotha, Grace Paul, Richard Shell, Lindsay N Alfano, Linda P Lowes, Anne M Connolly, Megan A Waldrop
{"title":"Transitioning From Nusinersen to Risdiplam for Spinal Muscular Atrophy in Clinical Practice: A Single-Center Experience.","authors":"Can Ebru Bekircan-Kurt, Sharmada Subramanian, Shannon Chagat, Samuel J Mackenzie, Megan Iammarino, Natalie Reash, Carson Richardson, Chang-Yong Tsao, Garey Noritz, Courtney Gushue, Kavitha Kotha, Grace Paul, Richard Shell, Lindsay N Alfano, Linda P Lowes, Anne M Connolly, Megan A Waldrop","doi":"10.1002/mus.28329","DOIUrl":"10.1002/mus.28329","url":null,"abstract":"<p><strong>Background: </strong>Nusinersen and risdiplam are U.S. Food and Drug Administration (FDA)-approved treatments for spinal muscular atrophy (SMA). No head-to-head clinical trials to assess efficacy exist. Observational studies are needed to determine if transitioning to risdiplam is safe and efficacious.</p><p><strong>Methods: </strong>This retrospective study at Nationwide Children's Hospital included individuals with SMA treated with nusinersen who switched to risdiplam. Motor, pulmonary and bulbar function were assessed before and 2 years after nusinersen and risdiplam initiation.</p><p><strong>Results: </strong>Forty-four individuals were included: 11 with SMA type 1, 25 with SMA type 2 and 8 with SMA type 3. Motor function improved after initiation of nusinersen treatment with the most significant improvements seen in the first year. After transition to risdiplam, motor function remained largely stable. Need for noninvasive ventilation (NIV) overnight occurred in both groups. Cough peak flow significantly improved in the risdiplam group. Hospitalizations were the same in both groups. One individual in the nusinersen group gained the ability to take some food by mouth; two individuals in the risdiplam group achieved some oral feeding and two became exclusively orally fed.</p><p><strong>Conclusions: </strong>As expected, motor function was most improved in treatment naïve individuals in the first year after nusinersen initiation. Over half of our study population had posterior spinal fusion surgery (57%) which significantly impacted motor and respiratory outcomes, though slightly less so in the risdiplam group. Overall, our data demonstrates that transitioning from nusinersen to risdiplam is associated with a favorable safety profile and stable motor outcomes.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"414-421"},"PeriodicalIF":2.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11799403/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142915296","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}
Muscle & NervePub Date : 2025-03-01Epub Date: 2024-12-24DOI: 10.1002/mus.28323
Jaclyn Tamaroff, Nicholas Joy, Bruce Damon, Larry W Markham, Thomas Donnelly, Karry Su, Maciej S Buchowski, Kimberly Crum, James C Slaughter, Meng Xu, W Bryan Burnette, Jonathan Soslow
{"title":"Physical Activity Correlates With Skeletal Muscle MRI Findings in Individuals With Duchenne Muscular Dystrophy.","authors":"Jaclyn Tamaroff, Nicholas Joy, Bruce Damon, Larry W Markham, Thomas Donnelly, Karry Su, Maciej S Buchowski, Kimberly Crum, James C Slaughter, Meng Xu, W Bryan Burnette, Jonathan Soslow","doi":"10.1002/mus.28323","DOIUrl":"10.1002/mus.28323","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Skeletal muscle magnetic resonance imaging (MRI) is a validated noninvasive tool to assess Duchenne muscular dystrophy (DMD) progression. There is interest in finding DMD biomarkers that decrease the burden of clinical trial participation, such as wearable devices. Our aim was to evaluate the relationship between activity, via accelerometry, and skeletal muscle MRI, particularly T<sub>2</sub> mapping.</p><p><strong>Methods: </strong>DMD children and young adults completed skeletal muscle MRI and were asked to wear an accelerometer on the dominant wrist for 7 days. MRI data included fat-suppressed transverse relaxation time (T<sub>2</sub>) mapping of the calves and longitudinal relaxation time (T<sub>1</sub>) mapping. Activity was assessed as vector magnitudes (VMs) and fraction of time (FOT) in activity groups (sedentary 1 or 2, low 1 or 2, moderate-to-vigorous physical activity (MVPA)).</p><p><strong>Results: </strong>Participants (n = 22; median age 11.4 years, 41% ambulatory) wore the accelerometer for a median of 7 days. Longer T<sub>2</sub> in multiple lower extremity muscles was negatively correlated with VMs per minute (tibialis posterior Spearman's rho = -0.68, p < 0.001), even when accounting for age, ambulatory status, or glucocorticoid use. Longer T<sub>2</sub> of the tibialis posterior was positively correlated with FOT in sedentary 1 (rho = 0.49, p = 0.02) and negatively correlated with FOT in higher activity levels (low 1 (rho = -0.58, p = 0.004), low 2 (rho = -0.67, p = 0.002), MVPA (rho = -0.7, p < 0.001)).</p><p><strong>Discussion: </strong>In individuals with DMD, longer T<sub>2</sub> on skeletal muscle MRI of the calves moderately correlated with lower activity levels indicating the potential use of home accelerometry as a future clinical trial biomarker of skeletal muscle health and progression in DMD.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"353-359"},"PeriodicalIF":2.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11799400/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142885879","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":"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}
Muscle & NervePub Date : 2025-02-13DOI: 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}
Muscle & NervePub Date : 2025-02-12DOI: 10.1002/mus.28370
Christi M Lero, Annabelle Yang, Elyse Everett, Kyle A Pitzer, Kelly McCoy Gross, Karla T Washington
{"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":"https://doi.org/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":""},"PeriodicalIF":2.8,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","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":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Muscle & NervePub Date : 2025-02-12DOI: 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}