Muscle & Nerve最新文献

{"title":"The Role of Electrophysiology and Ultrasonography in Guiding Early Surgical Management of Traumatic Upper Limb Pediatric Nerve Injuries: A Case Series.","authors":"Haneen Abed, Pavandeep K Virdee, Manigandan Thygarajan, Andrew Lawley, Andrea Jester","doi":"10.1002/mus.28488","DOIUrl":"10.1002/mus.28488","url":null,"abstract":"<p><strong>Introduction/aims: </strong>There is a lack of clinical guidance regarding when to offer surgical exploration of traumatic nerve injuries in pediatric populations, with clinical examination, electrophysiology, and neuroimaging used variably to guide clinical decisions. This retrospective case series aimed to review the role of electrophysiology and ultrasound in guiding early surgical decisions.</p><p><strong>Methods: </strong>This is a retrospective case series over a 4-year period at Birmingham Children's Hospital. All patients aged 16 years or under presenting with traumatic upper limb injuries who underwent electrophysiology and ultrasound investigations were included.</p><p><strong>Results: </strong>The most common cause of traumatic nerve injury in this series was supracondylar fracture, with the ulnar nerve being most commonly injured. Initial electrophysiology and ultrasound findings showed concordance regarding the presence of nerve continuity in most cases, although initial findings were discordant in 3 out of 17 patients. In 14 patients, initial electrophysiology was consistent with incomplete axonotmetic nerve injury. Four patients showed possible neurotmesis. In two instances, initial US findings suggested nerve discontinuity with complete disruption of fascicular architecture, although in both cases, repeat imaging demonstrated the formation of a neuroma in continuity. Three out of 17 patients required surgical intervention, with 2 undergoing neurolysis and 1 nerve grafting.</p><p><strong>Discussion: </strong>This study demonstrates high concordance between electrophysiology and ultrasound in the initial assessment of traumatic pediatric upper limb peripheral nerve injuries. Results suggest a combination of these investigations may provide better diagnostic accuracy to guide initial management decisions and may also prevent an initial overestimation of injury burden.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"647-652"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144961885","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":"Sensitivity of Repetitive Nerve Stimulation Performed in Early and Later Stages of Myasthenia Gravis.","authors":"Morgan E Heber, Jeremy Hill, Janice M Massey, Vern C Juel, Shruti M Raja, Yuebing Li, Donald B Sanders","doi":"10.1002/mus.28486","DOIUrl":"10.1002/mus.28486","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Repetitive nerve stimulation (RNS) is a valuable tool in the diagnostic evaluation of myasthenia gravis (MG). However, there are conflicting reports of its sensitivity when performed ≤ 30 days from symptom onset. We aim to compare the sensitivity of RNS performed in the early versus later stages of MG.</p><p><strong>Methods: </strong>Data from patients with MG who underwent RNS testing from 2017 to 2021 at Cleveland Clinic and from 1975 to 2001 at Duke University Medical Center were reviewed.</p><p><strong>Results: </strong>A total of 525 patients with MG underwent RNS testing. RNS was performed ≤ 30 days after symptom onset in 32 patients (Early RNS) and in 493 patients > 30 days after onset (Late RNS). RNS was abnormal in 56.3% of patients in the Early RNS group and 59.4% of those in the Late RNS group (p = 0.72). There was no difference in RNS positivity rates for either group between the institutions.</p><p><strong>Discussion: </strong>We found no significant difference in the sensitivity of RNS performed within or after 30 days from the onset of MG. Our findings support the use and value of RNS in the diagnostic evaluation of MG in patients presenting both early and late.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"643-646"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144962213","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":"Predictors of Outcome in Myasthenic Crisis.","authors":"Ahmad R Abuzinadah, Mohammed H Alanazy, Nadeem Shafique Butt, Ziad H Alzabidi, Marwa L Alrehaili, Laila Alqahtani, Areej Abdulrahman Bushnag, Hussien Salem Alkully, AlBaraa Ali Beck, Seraj Makkawi, Alaa Maglan, Alanood Alsolaihim, Ali Alshehri, Mossaed Mohammed Alyahya, Muteb Khidhran Alotaibi, Shahad Jamal Aljasir, Aysha A Alshareef, Ahmed K Bamaga, Ahmad Abdulaziz Abulaban","doi":"10.1002/mus.28475","DOIUrl":"10.1002/mus.28475","url":null,"abstract":"<p><strong>Introduction/aims: </strong>The impact of maintenance steroid sparing immunosuppression (SSIS) and prednisone dosage on myasthenic crisis (MC) outcomes and their effects on the development of sepsis have not been well described. This study aimed to investigate the predictive value of these variables on MC outcomes.</p><p><strong>Methods: </strong>This retrospective, multi-center study examined the potential predictors of MC outcomes using univariate and multivariate analyses through mixed-effect logistic regression and Cox proportional hazards regression.</p><p><strong>Results: </strong>We included 57 MCs that occurred in 52 patients with a median age of 55 years (interquartile range [IQR]: 33-66), 26 of whom (45.6%) were male. Multivariate analysis showed that sepsis was associated with prolonged length of intensive care unit (ICU) stay (hazard ratio [HR] for time to discharge from the ICU: 0.27, 95% confidence interval [CI]: 0.11-0.71) and duration of ventilation (HR for time to extubation: 0.28, 95% CI: 0.12-0.67). Predictors of sepsis development were age > 50 years (odds ratio [OR]: 7.74, 95% CI: 1.29-46.53) and prednisone use of ≥ 60 mg/day during ICU stay (OR: 13.07, 95% CI: 2.17-86.73). The presence of maintenance SSIS at MC onset predicted a shorter length of ICU stay (HR for time to discharge from the ICU: 2.23, 95% CI: 1.13-4.42) and duration of ventilation (HR for time to extubation: 2.73, 95% CI: 1.37-5.43).</p><p><strong>Discussion: </strong>The presence of maintenance SSIS at MC onset and the prevention of sepsis are measures that could improve MC outcomes. The use of lower doses (< 60 mg/day) of prednisone, when feasible, could mitigate the risk of sepsis in MC.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"636-642"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144691032","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":"Systemic Neisseria sicca Infection Associated With Ravulizumab Therapy in Myasthenia Gravis.","authors":"McKenzye M DeHart McCoyle, Iryna M Muzyka, Suraj Muley, Benn Smith, Alec Johnson, Sandhya R Nagarakanti","doi":"10.1002/mus.28454","DOIUrl":"10.1002/mus.28454","url":null,"abstract":"","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"676-677"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144285450","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":"Transitions From Pediatric to Adult Neuromuscular Care: A Practical Guide.","authors":"Cynthia Wozow, Kathryn Mosher","doi":"10.1002/mus.28468","DOIUrl":"10.1002/mus.28468","url":null,"abstract":"<p><p>As many as 90% of individuals with childhood onset diseases now live into adulthood. Due to this increase in lifespan, many, such as those with childhood onset neuromuscular disorders (NMDs), need health care transition to adult primary and specialty care providers. Despite growing recognition of the need for transition, only 17% of youth with special healthcare needs are receiving assistance with health care transition. One primary model of transition is the six core elements of transition as defined by the National Alliance to Advance Adolescent Health in their \"Got Transition\" which serves as a primary framework for other models of care. Growing evidence suggests continued barriers to the transition process, including difficulty finding adult specialists, lack of sufficient communication between pediatric and adult transition care providers, insurance issues, and lack of family/patient engagement in the process. Additionally, there are characteristics of the care team members (patient, family, pediatric and adult healthcare providers) themselves that serve as either positive or negative predictors of the success of transition, such as patient level of disability, family knowledge of the disease process, education level, adherence to treatment, investment in transitioning care, and provider recognition of limits of their own scope of practice. Use of tools to assess patient and family readiness to begin the transition process, optimization of the electronic medical record (EMR) to share information, telehealth, and dedicated transition clinics are key factors in making this process smoother for both patients and healthcare providers.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"558-565"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144732390","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":"3D Vascular Analysis of Regenerative Peripheral Nerve Interfaces Shows Neovasculature in Muscle Core by Day 5 Post Surgery.","authors":"Alexis L Lowe, Maria Issa, Johnnie A Johnson, Dian Chen, Ethan Brown, Alessandro Ascani Orsini, Kiara N Quinn, Akanksha Bhargava, Arvind Pathak, Sami Tuffaha, Nitish V Thakor","doi":"10.1002/mus.70001","DOIUrl":"10.1002/mus.70001","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Regenerative peripheral nerve interfaces (RPNIs) are skeletal muscle autografts that can be used as \"bio-amplifiers\" of nerve signals in bionic systems. An RPNI may die if its tissue does not revascularize properly; it is not known exactly how neovascular patterns are established in grafts immediately following surgery. Our research goal was to elucidate this process in 3D to provide insight for improving implantable interface design for RPNIs.</p><p><strong>Methods: </strong>Rats received unilateral RPNI surgery using the soleus muscle, and 5 or 10 days post surgery, they received ultrasound imaging of both hindlimbs before sacrifice. Tissue was harvested for micro-CT imaging of vessels and histology.</p><p><strong>Results: </strong>Total tissue volume of the RPNIs was significantly reduced relative to contralateral naïve soleus muscles from Day 5 to Day 10, indicating gradual atrophy (p < 0.01). RPNI groups on both days had core vascular fractions around 12% compared to ~17% in naïve control muscles. There was an unexplained change in the distribution of vessels from the distal end of the RPNI to the proximal end, with the vascular density increasing from Day 5 to Day 10 in the proximal end (p = 0.02), but not in the distal end.</p><p><strong>Discussion: </strong>We expected the vascular fraction within the RPNIs' core would increase from Day 5 to Day 10 post surgery; but that was not observed via micro-CT. The change in vessel density from end to end of the RPNIs over time might be influenced by the attachment site of the muscle to the transected nerve.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":"72 4","pages":"662-671"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145065338","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":"Muscle MRI Changes in Nebulin-Related Nemaline Myopathy.","authors":"Yajie Wang, Yun Yuan, Zhiying Xie, Yanjuan Wang, Yakun Wu, Meng Yu","doi":"10.1002/mus.28465","DOIUrl":"10.1002/mus.28465","url":null,"abstract":"<p><strong>Introduction/aims: </strong>NEB mutations are the most frequent causes of autosomal recessive subtypes of nemaline myopathies (NMs). There are few studies on muscle magnetic resonance imaging (MRI) changes in NEB-related NMs. We aimed to characterize the diagnostic and prognostic value of muscle MRI patterns in NEB-related NMs.</p><p><strong>Methods: </strong>Twenty-one patients with confirmed diagnoses of NEB-related NMs were enrolled. The relationships between MRI changes in lower extremity muscles and clinical features were investigated.</p><p><strong>Results: </strong>Most patients (18/21) presented with lower limb muscle weakness, with a distal predominance. In the lower limbs, the mean fatty infiltration score was greatest in the soleus (2.44), followed by the gastrocnemius (2.00) and gluteus maximus (2.05). The severity of fatty infiltration in the thigh muscles was correlated with age at the time of MRI (r = 0.569, p = 0.009) and disease duration from symptom onset to MRI (r = 0.597, p = 0.005). The mean fatty infiltration scores of the gluteus maximus, vastus medialis, sartorius, and adductor maximus were moderately correlated with disease duration (r-values = 0.467-0.597).</p><p><strong>Discussion: </strong>Our study revealed that the degree of lower limb muscle fatty infiltration in NEB-related NMs was correlated with the disease course, with the gluteus maximus of the thigh and soleus of the calf being the most severely affected. Edema of the lower limb muscles was also more common and noticeable in the calf than in the thigh. These muscle MRI changes may be helpful in the diagnosis and follow-up of this disease.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"580-585"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144529002","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":"Clinical Utility of Far-Field Potentials in Amyotrophic Lateral Sclerosis.","authors":"Aicee Dawn Calma, Nathan Pavey, Claudia Santos Silva, Yukiko Tsuji, Mehdi A J Van den Bos, Michelle A Farrar, Parvathi Menon, Steve Vucic","doi":"10.1002/mus.28480","DOIUrl":"10.1002/mus.28480","url":null,"abstract":"<p><strong>Introduction/aims: </strong>Far field potentials (FFP) have been proposed as a reliable neurophysiological prognostic biomarker in amyotrophic lateral sclerosis (ALS). This study evaluates the diagnostic utility of ulnar nerve FFP in ALS.</p><p><strong>Methods: </strong>Comprehensive peripheral neurophysiological assessments were conducted in 62 ALS and 43 ALS-mimicking disorder participants. The ulnar nerve was stimulated at the wrist, recording motor responses over the abductor digit minimi (ADM) muscle. Conventional compound muscle action potentials (CMAP), FFP, and near field potential amplitudes were recorded, alongside the split-hand index, neurophysiological index, motor unit number estimation (MScanFit-MUNE), and motor unit index (MUNIX). Diagnostic utility was evaluated using receiver operating characteristic (ROC) analysis.</p><p><strong>Results: </strong>In ALS, FFP amplitude was significantly lower (5.07 ± 0.36 mV) compared to ALS mimics (8.25 ± 0.40 mV, p < 0.001). FFP amplitude exhibited a moderate-to-strong correlation with neurophysiological biomarkers, including CMAP amplitude (ρ = 0.77, p < 0.001), split-hand index (ρ = 0.53, p < 0.001), neurophysiological index (ρ = 0.52, p < 0.001), MUNIX (ρ = 0.69, p < 0.001), and MScanFit-MUNE (ρ = 0.66, p < 0.001). Weak-to-moderate correlations were also observed with clinical measures of disease progression, including upper limb muscle strength, ALS functional rating score-revised (ALSFRS-R) and the rate of decline in the ALSFRS-R fine motor subscore. ROC analysis demonstrated that FFP amplitude reliably distinguished ALS from mimicking disorders (AUC = 0.80, 95% CI: 0.71-0.89), with consistent diagnostic accuracy across ALS phenotypes.</p><p><strong>Discussion: </strong>The diagnostic capability of FFP amplitude was comparable to established neurophysiological biomarkers utilized in ALS. It is a promising prognostic and diagnostic biomarker for ALS. Its simplicity and reproducibility complement traditional neurophysiological measures, offering potential for clinical application in ALS diagnosis and monitoring.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"616-624"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12435138/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144659659","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":"Elevated Cancer Prevalence Identified at Specific Anatomical Sites Among People With Myotonic Dystrophy Using a Population-Based Sample.","authors":"Wanfang Zhang, Vinay Bhandaru, Nicholas E Johnson, Amy Moore, Abigail Lyons, Reba Berry, Christina Westfield, Daria McMahon, Aida Soim, Bo Cai, Suzanne McDermott","doi":"10.1002/mus.28464","DOIUrl":"10.1002/mus.28464","url":null,"abstract":"<p><strong>Introduction/aim: </strong>To optimize patient care and cancer screening in myotonic dystrophy (DM), it is crucial to clarify cancer risks by DM type to guide targeted screening and prevention efforts. This study describes the prevalence of cancer among individuals diagnosed with DM from the Muscular Dystrophy Surveillance, Tracking, and Research Network (MD STARnet), and compares it to cancer prevalence in the US Surveillance, Epidemiology, and End Result (SEER) data.</p><p><strong>Methods: </strong>This retrospective cohort study used 2008-2019 medical records data on 1229 individuals diagnosed with DM (DM1: 81.4%, DM2: 9.4%, DM-not otherwise specified [NOS]: 9.2%) from MD STARnet. Age- and sex-specific cancer prevalence rates from SEER were applied to the MD STARnet data to calculate 12-year limited duration (LD) and complete standardized prevalence ratios (SPR) for total cancer and by primary cancer site.</p><p><strong>Results: </strong>Over a 12-year span, individuals with DM1 had a 4.01-fold higher LD prevalence of thyroid cancer and a 17.97-fold higher LD prevalence of nonmelanoma skin cancer compared to the SEER prevalence, respectively. Complete prevalence assessments identified that individuals with DM1 had a 4.18-fold higher prevalence of thyroid cancer, a 2.36-fold higher prevalence of melanoma, and a 4.68-fold higher prevalence of ovarian cancer. For DM2, the study observed a 34.02-fold higher LD prevalence for nonmelanoma skin cancer. Additionally, a significantly elevated complete prevalence of breast cancer (2.65-fold) was noted for DM2 compared to SEER data.</p><p><strong>Discussion: </strong>Future research with larger cohorts is needed to define cancer risks by DM subtype and investigate underlying biological mechanisms.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"570-579"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144529000","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":"Analysis of Long-Term Function and Survival of Edaravone Oral Suspension-Treated Patients With Amyotrophic Lateral Sclerosis Using PRO-ACT Data as Historical Placebo Controls.","authors":"Fumihiro Takahashi, Angela Genge, Manabu Hirai, Daniel Selness, Vesna Todorovic, Art Wamil, Nissim Sasson, Stephen Apple, Yoshiteru Ushirogawa","doi":"10.1002/mus.28462","DOIUrl":"10.1002/mus.28462","url":null,"abstract":"<p><strong>Introduction/aims: </strong>On/Off dosing of intravenous (IV) edaravone and edaravone oral suspension was US Food and Drug Administration (FDA)-approved for Amyotrophic Lateral Sclerosis (ALS) treatment. Placebo-controlled trials showed that IV edaravone slows the rate of physical functional decline in patients with ALS. Here, the impact of edaravone oral suspension on function and survival was assessed.</p><p><strong>Methods: </strong>Edaravone oral suspension was investigated in clinical trials MT-1186-A01/A02/A03/A04. Patients from Studies MT-1186-A02/A04 (prespecified analysis) and Studies MT-1186-A01/A02/A03/A04 (post hoc analysis) were propensity score matched 1:1 on 10 baseline variables with historical Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) placebo patients (not receiving active investigational treatment in their trials) to assess the impact of edaravone oral suspension on function and survival.</p><p><strong>Results: </strong>In the prespecified analysis, 78 edaravone oral suspension-treated patients from Studies MT-1186-A02/A04 demonstrated a survival benefit versus 78 matched PRO-ACT placebo patients (p = 0.005). Baseline risk-adjusted hazard ratio showed an 84% decreased risk of death in edaravone oral suspension versus PRO-ACT placebo patients (p = 0.005). ALS Functional Rating Scale-Revised (ALSFRS-R) total score change from baseline at Week 48 was -8.4 points for edaravone oral suspension versus -14.1 points for PRO-ACT placebo patients (p < 0.001). In the post hoc analysis, patients from Studies MT-1186-A01/A02/A03/A04 (n = 210) propensity score matched to PRO-ACT placebo patients (n = 210) showed statistically significantly longer time to death and smaller ALSFRS-R change from baseline at Week 48; restricted mean survival time showed a 7.3-month improvement (p < 0.001).</p><p><strong>Discussion: </strong>This suggests edaravone oral suspension significantly increases survival time and decreases physical functional decline versus PRO-ACT placebo patients.</p>","PeriodicalId":18968,"journal":{"name":"Muscle & Nerve","volume":" ","pages":"586-596"},"PeriodicalIF":3.1,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12435165/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144540877","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}