Amyotrophic lateral sclerosis & frontotemporal degeneration最新文献

{"title":"Limited data capture on reproductive medicine use in amyotrophic lateral sclerosis: implications for monitoring access.","authors":"Shanice Allen, Jade Howard, Christopher J McDermott, Felicity Boardman, Alisdair McNeill","doi":"10.1080/21678421.2026.2618124","DOIUrl":"10.1080/21678421.2026.2618124","url":null,"abstract":"<p><p>There is very limited evidence around the use of reproductive genetic testing in individuals with amyotrophic lateral sclerosis (ALS)-linked gene variants. This study aimed to identify the use of reproductive genetic testing in these individuals to understand patterns of (under)utilization and to identify barriers to equitable access. Freedom of information requests were sent in January 2025 to the 22 regional clinical genetics centers across the UK around reproductive services for individuals with, or at risk for, ALS and Huntington's disease. Limited data were available with only six trusts answering in full. The data that our study yielded raises significant concerns and inconsistencies regarding clinical recording and reporting of reproductive genetic counseling and testing. The absence of standardized retrievable data limits the ability to assess utilization and may point toward a systemic issue in data capture of reproductive genetic services for individuals at risk of ALS, and by extension, those affected by other genetic conditions.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"608-610"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146115190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Timing of communication and technology control support in ALS - a systematic review.","authors":"Simon Judge, Kirsty Ballesteros, Christopher J McDermott, Steven Bloch","doi":"10.1080/21678421.2026.2627899","DOIUrl":"10.1080/21678421.2026.2627899","url":null,"abstract":"<p><p><i>Objective:</i> To review evidence on the optimal timing of interventions that support communication and technology control for people living with Amyotrophic Lateral sclerosis (ALS). <i>Methods:</i> A systematic review was conducted following a pre-registered protocol. Databases were searched for studies involving people living with ALS that addressed timing of assistive technology interventions for communication or technology control. Screening and data extraction were completed in duplicate, findings were synthesized using a thematic analysis, and relevant findings presented as a descriptive summary. <i>Results:</i> Twenty-eight studies met the inclusion criteria. Evidence focused overwhelmingly on communication support rather than wider assistive technology interventions. Need for a communication aid typically occurs between one and five years from diagnosis and the timing of this varies significantly according to the site of onset of ALS. There are significant variations in the timing of changes for individuals within these groupings and there are likely a larger number of groupings that would be clinically useful. A significant correlation between changes in speaking rate and intelligibility has been shown. Once changes to speech do start to occur then the time to the loss of functional speech appears relatively consistent across the types of ALS. <i>Conclusion:</i> Current best practice guidelines are not reflective of the findings of this review and do not support professionals in identifying how to provide timely support. Monitoring speech changes systematically may support timely intervention. There is potential for individual level predictive modeling to help support people living with ALS to be proactive and prepared for changes.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"504-512"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146260222","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Systemic immune-metabolic interactions in patients with amyotrophic lateral sclerosis: correlations of plasma cytokines with metabolic status.","authors":"Stephanie L Howe, Cory J Holdom, Pamela A McCombe, Robert D Henderson, Frederik J Steyn, Shyuan T Ngo","doi":"10.1080/21678421.2026.2627902","DOIUrl":"10.1080/21678421.2026.2627902","url":null,"abstract":"<p><strong>Objective: </strong>Amyotrophic lateral sclerosis (ALS) is a clinically heterogeneous disease influenced by multiple biological processes. Altered energy metabolism, particularly hypermetabolism, is observed in some people living with ALS (plwALS) and is associated with faster progression. The mechanisms underlying this metabolic phenotype remain unclear. Inflammation has been proposed as a contributing factor. We examined whether plasma cytokines, as markers of systemic inflammation, are associated with energy expenditure in plwALS.</p><p><strong>Methods: </strong>Plasma samples from 77 plwALS and 90 non-neurodegenerative controls were analyzed using a multiplex immunoassay quantifying 14 cytokines. Cytokine concentrations were compared between groups and examined in relation to body composition, resting energy expenditure, and functional capacity as inferred by the ALSFRS-R.</p><p><strong>Results: </strong>Interleukin-6 (IL-6) and monocyte chemoattractant protein-1 (MCP-1) concentrations were higher in plwALS than in controls prior to correction for multiple comparisons (p = 0.006; p.adj = 0.083, and p = 0.035; p.adj = 0.237, respectively). Hypermetabolism was more prevalent in the ALS cohort (36% compared to 11.5%, p < 0.001); however, cytokine concentrations were not significantly different across metabolic subgroups and showed no statistically significant association with metabolic index, energy expenditure, or functional measures.</p><p><strong>Conclusions: </strong>Findings confirm modest elevations in plasma levels of IL-6 and MCP-1 in ALS but provide no evidence that circulating cytokines correlate with hypermetabolism. Further studies integrating longitudinal and tissue-specific analyses are needed to clarify immune-metabolic mechanisms contributing to disease heterogeneity in ALS.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"581-591"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146195577","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cognitive and behavioral involvement in spinal and bulbar muscular atrophy (SBMA): a systematic review.","authors":"Giulia De Luca, Edoardo Nicolò Aiello, Arianna Moreschi, Beatrice Curti, Sonia Marcato, Federica Paredi, Federico Verde, Vincenzo Silani, Nicola Ticozzi, Barbara Poletti, Gianni Sorarù","doi":"10.1080/21678421.2025.2608228","DOIUrl":"10.1080/21678421.2025.2608228","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed at reviewing currently available evidence on cognitive and behavioral involvement in spinal and bulbar muscular atrophy (SBMA).</p><p><strong>Methods: </strong>This review was performed in accordance with the 2020 PRISMA guidelines. We included case reports/series and group studies reporting data on the neuropsychological features of SBMA patients.</p><p><strong>Results: </strong>Out of 123 unique records identified, 11 studies were included (<i>N</i> = 5 case reports/series; <i>N</i> = 6 group studies), comprising 151 SBMA patients. Among case reports and series, 6 out of 10 patients were reported to exhibit some degree of neuropsychological involvement, with two being diagnosed with a dementia condition resembling the characteristics of behavioral variant-frontotemporal dementia. Three of the 6 case-control studies found no cognitive differences between SBMA patients and healthy controls. Cognitive deficits reported in group studies mostly involved the executive, attentional, memory and visuo-spatial/-constructive domains.</p><p><strong>Conclusions: </strong>The available evidence on cognitive and behavioral involvement in SBMA is scarce and conflicting. However, it is conceivable that at least some patients may exhibit dysexecutive-type changes, possibly reflecting a frontal system involvement.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"652-670"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147461358","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ALSUntangled #82: N-acetylcysteine.","authors":"Sandeep Yerraguntla, Bhavya Bakshi, Keshav Chandran, Juliette Foucher, Michael Benatar, Paul Wicks, Richard Bedlack, David Shirilla, Yuyao Sun, Nicholas Maragakis, Ethan Greenstein, Javier Mascias Cadavid, Anusha Rao, Olivia Allen, Kara Dyckman, Olivia Wang, Morgan Beauchamp, Vincent Chang, Andrew Brown, Olimpia Carbunar, Sabrina Paganoni, Tulio Bertorini, Erik Pioro, Basil Elsharif, Nan Jiang, Gary Pattee, Gregory Carter, Sarah Breevoort, Elia Tito, George Nathaniel, Carlayne Jackson, Natasha Olby, Christopher McDermott, Dylan Ratner, Xiaoyan Li","doi":"10.1080/21678421.2026.2638590","DOIUrl":"10.1080/21678421.2026.2638590","url":null,"abstract":"<p><p>N-acetylcysteine is a thiol-containing compound and a precursor of glutathione, with mechanistic plausibility for ALS, including reducing oxidative stress, regulating neuroinflammation, and mitigating mitochondrial dysfunction. Preclinical studies have yielded conflicting results on whether N-acetylcysteine can delay the onset of motor impairment and prolong survival in ALS mouse models. Several case studies of oral or subcutaneous administration of N-acetylcysteine in patients with ALS did not demonstrate convincing benefits. Clinical trials to date have also failed to demonstrate efficacy in slowing ALS progression. While N-acetylcysteine shows theoretical promise, further research is needed to clarify its therapeutic role in ALS. At present, ALSUntangled does not support the use of N-acetylcysteine as a treatment to slow ALS progression.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"717-721"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147349811","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development of a machine learning-based survival prediction model for ALS inclusive of the advanced-stage population.","authors":"Danielle Beaulieu, Kelly Smith, Campbell Ross, Sylvia Yip, Tania C Felizardo, Christina Fournier, Jonathan D Glass, James D Berry, Daniel H Fowler, David L Ennist","doi":"10.1080/21678421.2026.2652322","DOIUrl":"10.1080/21678421.2026.2652322","url":null,"abstract":"<p><strong>Objective: </strong>Develop a machine learning-based model for survival prediction in ALS, including advanced-stage patients (≤50% predicted normal vital capacity [VC₅₀]).</p><p><strong>Methods: </strong>Training data from the PRO-ACT Database (<i>n</i> = 6896) was supplemented with advanced-stage ALS patients (<i>n</i> = 678), with model validation on distinct advanced-stage ALS patients (<i>n</i> = 403). Baseline patient characteristics, including slopes from symptom onset, were used to train a random forest model to identify parameters with the greatest relative importance (RI) for predicting survival outcomes. These parameters were used to train a gradient-boosting machine (GBM) model that generated patient-level survival predictions (log-hazard). Model discrimination and calibration were quantified by C-index and calibration-in-the-large plus calibration slope, respectively. Kaplan-Meier curves were generated, with patient stratification into tertiles based on the predicted survival risk score.</p><p><strong>Results: </strong>Baseline characteristics with the highest RI for driving survival predictions included: VC% slope (20.2%); age (12.4%); VC% (9.9%); VC(L) (7.5%); ALSFRS-R (6.6%); and ALSFRS-R slope (5.1%). Model performance upon external validation was satisfactory for both discrimination (C-index, 0.709 [95% CI, 0.671-0.746]) and calibration (calibration-in-the-large, 0.083 [95% CI, -0.073-0.232]; calibration slope, 0.992 [95% CI, 0.789-1.198]). At 8-months from baseline, the model successfully stratified patients by survival prognosis, with low-, average-, and high-risk population tertiles having observed median survival probabilities of 85, 69, and 43%, respectively.</p><p><strong>Conclusions: </strong>This model accurately predicts survival prognosis in ALS, including patients with severely impaired respiratory function. This new understanding of patient-specific factors that drive survival prognostication will be invaluable for reducing patient heterogeneity in clinical trials evaluating novel therapeutic modalities in early- and advanced-stage ALS.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"678-688"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13138003/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147679234","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Integrating administrative health data and machine learning to predict ALS onset.","authors":"Toni Mora, David Roche, Pol Andrés Benito, Alejandro Caravaca Puchades, Mónica Povedano","doi":"10.1080/21678421.2025.2596691","DOIUrl":"10.1080/21678421.2025.2596691","url":null,"abstract":"<p><strong>Background: </strong>This study aims to develop a Machine Learning (ML) model to predict the initial diagnosis of Amyotrophic Lateral Sclerosis (ALS).</p><p><strong>Methods: </strong>To predict ALS, a stacked model combining four ML algorithms-logistic Regression, Decision Tree, Random Forest, and Extreme Gradient Boosting-was implemented. The analysis utilized healthcare administrative data from Catalonia, encompassing 2,924,590 elderly individuals from 2014 to 2021, which were linked to socioeconomic factors and medication records.</p><p><strong>Results: </strong>The stacked model successfully predicted first-time ALS diagnoses, achieving an AUC of 0.86, with an accuracy of 0.86, specificity of 0.88, and sensitivity of 0.84. The most influential predictors included immunization encounters, South American origin, general medical and special examinations, hypertensive heart disease, and counseling. Other relevant features were sciatica, heart failure, liver metastases, healthcare use patterns, and chronic conditions such as hypertension, kidney disease, and hypercholesterolemia. These features reflect early clinical symptoms and healthcare usage patterns relevant to ALS detection.</p><p><strong>Conclusions: </strong>Machine Learning models, particularly stacked approaches, show promising results in predicting ALS diagnoses using administrative health data. Continued research is necessary to improve detection strategies and support their integration into healthcare systems.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"671-677"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145679521","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Targeting metabolic dysfunction in amyotrophic lateral sclerosis: therapeutic potential of GLP-1 receptor agonists.","authors":"Mohamed Mohsen Helal, Nereen A Almosilhy, Dina Essam Abo-Elnour, Rana Suhail Younis Jaffal, Eman Gomaa Allam, Mohammed A Almosilhy, Suhel F Batarseh, Mostafa Meshref","doi":"10.1080/21678421.2026.2627901","DOIUrl":"10.1080/21678421.2026.2627901","url":null,"abstract":"<p><p>Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive motor neuron loss and profound systemic metabolic dysfunction, including hypermetabolism, weight loss, insulin resistance, and altered glucose and lipid homeostasis. Increasing recognition of these metabolic abnormalities has driven interest in repurposing antidiabetic therapies, particularly glucagon-like peptide-1 (GLP-1) and GLP-1 receptor agonists (GLP-1RAs), for ALS. Beyond their established metabolic actions, GLP-1RAs exert pleiotropic effects relevant to neurodegeneration, including modulation of neuroinflammation, mitochondrial function, oxidative stress, excitotoxicity, and cell-survival signaling, with selected agents demonstrating central nervous system penetration. This narrative review summarizes current knowledge on metabolic impairment in ALS and critically evaluates the mechanistic rationale, preclinical evidence, and emerging clinical data supporting or opposing the use of GLP-1-based therapies in this disease. Preclinical studies suggest that GLP-1 signaling can provide neuroprotective and neurotrophic effects in ALS models, although findings are heterogeneous and highly dependent on compound selection, delivery strategy, and experimental design. In contrast, available clinical evidence is limited and does not demonstrate therapeutic benefit in ALS, while raising important safety concerns, particularly related to weight loss, lean mass reduction, and altered glucose regulation, factors associated with a worse prognosis in ALS. Collectively, current data indicate that although GLP-1-based therapies may have compelling biological plausibility and beneficial effects in other neurodegenerative disorders (NDGs), their role in ALS remains uncertain and potentially harmful. Well-designed, ALS-specific clinical studies are required to clarify safety, efficacy, and patient selection before GLP-1RAs can be considered for therapeutic use in this vulnerable population.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"513-539"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146183656","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ALS motor phenotypes: a revised 'OPM' classification.","authors":"Thomas Meyer, Nicola Ticozzi, Markus Weber, John Ravits, Paul Lingor, Magdalena Kuźma-Kozakiewicz, Matthias Boentert, Torsten Grehl, Philippe Corcia, Mónica Povedano Panadés, André Maier, Caroline Ingre, Hakan Cetin, Patrick Weydt, Christian Lunetta, Leonard van den Berg, Albert C Ludolph, David Brenner, Martin R Turner, Angela Genge","doi":"10.1080/21678421.2026.2644277","DOIUrl":"10.1080/21678421.2026.2644277","url":null,"abstract":"<p><strong>Background: </strong>Defining motor phenotypes in amyotrophic lateral sclerosis (ALS) is important for individualized care and optimal therapeutic trial design. The \"ALS-OPM\" classification is based on the onset region (O), the propagation of motor symptoms (P), and the degree of clinical upper (UMN) and/or lower (LMN) motor neuron dysfunction (M).</p><p><strong>Methods: </strong>An international ALS expert focus group was held in September 2025, followed by a consensus process through which revisions of the OPM classification were finalized.</p><p><strong>Results: </strong>Onset (O1-4) identifies first motor symptoms as relating to the head (O1), distal/proximal arm (O2d/p), respiratory/axial trunk (O3r/a), or distal/proximal leg (O4d/p). Onset symptoms are defined by weakness or slowed, poorly coordinated voluntary movements in the muscles of the head, arm, trunk, or leg, including dysarthria, dysphagia, dysphonia, dyspnea, and axial instability. Propagation (P1(n)) or absence of propagation (P0(n)) of motor symptoms from the onset region to another body region are designated, where <i>n</i> denotes the number of months from onset to propagation or assessment. The degree of UMN dysfunction (slowed, poorly coordinated voluntary movements, hyperreflexia and/or spastic muscle tone, emotional lability) and/or LMN dysfunction (weakness with associated muscle atrophy) is classified as follows: balanced UMN and LMN dysfunction (M0); dominant (M1d) or pure UMN dysfunction (M1p); dominant (M2d) or pure LMN dysfunction (M2p); and dissociated UMN/LMN dysfunction (M3), in which the arms and legs predominantly show LMN and UMN involvement, respectively.</p><p><strong>Conclusion: </strong>The revised ALS-OPM classification aims to make it routine, practical and feasible to capture phenotype in clinical practice and therapeutic trials.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"540-552"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147476368","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessment of language and executive functions in ALS: the brief executive Language screen.","authors":"Gail A Robinson, Mia R Phillips, Kristina Horne, Amelia Ceslis, Pamela A McCombe, Robert D Henderson","doi":"10.1080/21678421.2025.2607398","DOIUrl":"10.1080/21678421.2025.2607398","url":null,"abstract":"<p><p><i>Objective</i>: Language and executive functioning are two domains commonly impacted in ALS and should be assessed sensitively and briefly. This paper investigates the utility of the Brief Executive Language Screen (BELS) in ALS. <i>Methods</i>: ALS patients (<i>N</i> = 27) were compared to age, education, and pre-morbid intelligence-matched healthy controls (<i>N</i> = 91) at the group level using ANCOVA and t-tests. A case series was also conducted to explore individual and subgroup performance on the BELS and Edinburgh Cognitive and Behavioral ALS Screen (ECAS). <i>Results</i>: Groups were largely matched on neuropsychology baseline measures. The ALS group performed significantly worse on BELS Propositional Language and Executive Function subtests, and on overall BELS scores. Group results suggest setting a goal can increase phonemic fluency and spontaneous speech output, after controlling for motor speed. The case series revealed almost half of patients (across all subgroups) were impaired on the BELS, compared to 15% impaired on the ECAS (patients on the more severe end of the ALS-FTD spectrum). <i>Conclusions</i>: The BELS rapidly assesses language and executive functions, and provides valuable information for management of cognitive difficulties (i.e., goal setting), which can help improve or maintain conversational speech. The BELS may help to identify subtle impairments that may otherwise go undetected.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"641-651"},"PeriodicalIF":2.8,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145991937","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}