Amyotrophic lateral sclerosis & frontotemporal degeneration最新文献

{"title":"Differentiating upper- and lower motor neuron diseases using automated acoustic analysis.","authors":"Justin Truong, Leif Simmatis, Timothy Pommée, Agessandro Abrahao, Kerry Adams, Marvin Chum, Colleen O'Connell, Angela Genge, Sanjay Kalra, Benjamin Ritsma, Kerri Schellenberg, Christen Shoesmith, Lorne Zinman, Gord Jewett, Yana Yunusova","doi":"10.1080/21678421.2025.2506444","DOIUrl":"10.1080/21678421.2025.2506444","url":null,"abstract":"<p><strong>Objective: </strong>Motor neuron diseases (MNDs) result in a spectrum of motor impairments, including considerable effects on speech function, which manifest as dysarthria-a motor speech disorder. Speech metrics are increasingly recognized as critical biomarkers with potential utility in disease diagnosis and phenotyping. This study aimed to (1) characterize acoustics of upper motor neuron (UMN) and lower motor neuron (LMN) dysarthria presentations in MNDs, and (2) identify relationships between bulbar disease severity scores and acoustic features, as these could collectively enable personalized approaches to management of these diseases.</p><p><strong>Methods: </strong>Data from 16 individuals with primary lateral sclerosis (PLS) representing UMN disease, 14 individuals with spinal and bulbar muscular atrophy (SBMA) representing LMN disease, and 25 neurologically healthy individuals were analyzed. Clinical measures were also collected from PLS and SBMA groups. All participants were remotely recorded performing passage reading, rapid syllable repetition, and vowel phonation. Fifty-two acoustic features were extracted representing articulation, phonation, prosody, resonance, and overall speech timing. Features were compared using Kruskal-Wallis tests for between-group comparisons and Spearman correlations between acoustic features and clinical scores.</p><p><strong>Results: </strong>Articulatory and prosodic features best differentiated PLS, SBMA and controls. Correlations were observed in the PLS group between the clinical score and various articulatory features, most notably those indexing tongue and jaw movements.</p><p><strong>Conclusions: </strong>Our study demonstrated that acoustic assessment could capture fingerprints of dysarthrias associated with PLS and SBMA. These findings also demonstrate the potential for remote speech assessment to characterize diverse dysarthria profiles and pave the way for creating ways for personalized disease management approaches in clinical care and trials.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"691-701"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12380423/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144200905","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":"How healthcare professionals support cough and secretion management in amyotrophic lateral sclerosis (ALS): a UK national survey.","authors":"Charlotte Massey, Alys Wyn Griffiths, Christopher McDermott, Esther Hobson","doi":"10.1080/21678421.2025.2522401","DOIUrl":"10.1080/21678421.2025.2522401","url":null,"abstract":"<p><strong>Objective: </strong>To understand the practices of healthcare professionals supporting people with Amyotrophic Lateral Sclerosis (ALS) to manage cough and secretion issues. This includes utilization of and confidence in assessment and treatment techniques and barriers and enablers of care.</p><p><strong>Methods: </strong>An online cross-sectional survey was distributed to UK healthcare professionals involved in cough and/or secretion management care in people with ALS.</p><p><strong>Results: </strong>A total of 113 responses were analyzed, over half were from physiotherapists (52%). The majority (71%) of respondents reported a role managing saliva and secretions. Just under two thirds (60%) routinely assessed cough and almost all (89%) routinely assessed secretions. Healthcare professionals reported reduced confidence assessing secretions compared with cough and very few (5%) used validated secretion outcome measures. Participants reported lower confidence implementing all treatment strategies than recommending them. Multiple barriers to care were identified, including access to specialist care and equipment, education and skills training and a lack of evidence-based care guidelines.</p><p><strong>Conclusion: </strong>Cough and secretion management is complex and involves numerous professional groups. There is a need for clinical and educational interventions that address knowledge and skill gaps in managing cough and secretion issues, which will help increase healthcare professionals' confidence in assessing and treating these complex problems.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"637-648"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144499592","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":"Self-care mobile application for people with Amyotrophic Lateral Sclerosis: a development and usability test.","authors":"Juyeon Oh, Hyeon Sik Chu","doi":"10.1080/21678421.2025.2507169","DOIUrl":"10.1080/21678421.2025.2507169","url":null,"abstract":"<p><strong>Objective: </strong>Mobile technology can significantly enhance self-care for individuals with amyotrophic lateral sclerosis (ALS). This study aims to develop and evaluate the usability of a mobile application that provides relevant information and manages disease-related data for ALS patients and their families.</p><p><strong>Methods: </strong>A mobile application compatible with Android and iOS platforms was developed following the four phases of the System Development Life Cycle. The content was derived from a literature review, stakeholder focus group interviews, and in-depth interviews with ALS patients, family members, and healthcare professionals. The final application was validated by experts (<i>n</i> = 7), tested for usability by ALS patients and caregivers (<i>n</i> = 18), and evaluated using the System Usability Scale (SUS) to assess effectiveness and user satisfaction.</p><p><strong>Results: </strong>The application includes features such as tailored health data visualization, symptom tracking, text-to-speech functionality, and access to information customized based on the overall the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised score, thereby supporting patient-centered care and daily disease management. The mean SUS score was 75.00 ± 9.57 for expert panel members and 63.75 ± 10.26 for the target users, indicating an acceptable level of usability.</p><p><strong>Conclusions: </strong>The mobile application was evaluated as practical, acceptable, and feasible for ALS patients and their caregivers, with positive feedback on its usability and potential to improve self-care management.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"683-690"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144103398","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":"Behavioral subtypes impact prognosis and survival in amyotrophic lateral sclerosis: a clustering-based approach.","authors":"Nga Yan Tse, Jashelle Caga, Rebekah M Ahmed, Srestha Mazumder, Chilan Nguyen, William Huynh, Anneliese Karjalainen, Hannah C Timmins, Eleanor Ramsey, Dayna-Lee Talbot, Glenda M Halliday, Matthew C Kiernan, Emma M Devenney","doi":"10.1080/21678421.2025.2522402","DOIUrl":"10.1080/21678421.2025.2522402","url":null,"abstract":"<p><strong>Background: </strong>Behavioral impairments are well established in amyotrophic lateral sclerosis (ALS). A refined understanding of the contribution of delineated patterns of behavioral impairments to prognosis is vitally important.</p><p><strong>Methods: </strong>Leveraging data-driven two-step cluster analysis, we first stratified a large cross-sectional cohort of 170 ALS patients into distinct phenotypic subtypes based on their baseline behavioral profiles, as determined by the well-validated and informant-rated Motor Neuron Disease Behavioral Instrument (MiND-B). Mixed-effects model and multivariate Cox regression analyses were performed to compare rate of functional decline as measured by the revised ALS functional rating scale (ALSFRS-R) over follow-up assessments in 121 participants, as well as survival duration (<i>n</i> = 130), between the behavioral subtypes. <i>Results:</i> Clustering analysis yielded three behavioral phenotypes characterized by <b>1)</b> intact behavioral functioning (<i>n</i> = 125), <b>2)</b> apathy alone (<i>n</i> = 20), and <b>3)</b> concurrent disinhibition and stereotypical behavior (<i>n</i> = 25). Apathy was associated with both significantly shorter survival (<i>p</i> = .003) and most rapid functional decline across follow-up assessments (both <i>p</i> <.001). Importantly, this pervasive effect was not observed in other behavioral cluster groups.</p><p><strong>Conclusions: </strong>Extending previous cross-sectional work, current findings offer delineation of the trajectory of clinical outcomes associated with classic behavioral phenotypes of ALS. Converging with past evidence of unique disease and progression profile in ALS patients with apathy, our work provides strong support for behavioral change and in particular apathy as a reliable indicator of poor prognosis across cross-sectional and longitudinal markers of clinical outcomes.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"729-738"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144980682","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":"Serum NfL, but not GFAP, differentiates primary lateral sclerosis from adrenomyeloneuropathy and hereditary spastic paraplegia type 4.","authors":"Christoph Kessler, Carlo Wilke, Holger Hengel, Tim W Rattay, Aleksandra Maleska Maceski, Jens Kuhle, Ludger Schöls, Rebecca Schüle","doi":"10.1080/21678421.2025.2557936","DOIUrl":"https://doi.org/10.1080/21678421.2025.2557936","url":null,"abstract":"<p><strong>Objective: </strong>Neurodegenerative upper motor neuron (UMN) syndromes ranging from primary lateral sclerosis (PLS) to pure and complicated types of hereditary spastic paraplegia (HSP) remain challenging to differentiate clinically, especially in the early stages of disease. As they share the hallmark of spastic paraparesis, easily accessible biomarkers are warranted to facilitate an early diagnosis.</p><p><strong>Methods: </strong>We examined serum neurofilament light chain (sNfL) and serum glial fibrillary acidic protein (sGFAP) as diagnostic biomarkers to differentiate PLS from HSP, represented by two paradigmatic subtypes: SPG4, the most common type of pure HSP, and adrenomyeloneuropathy (AMN), a common complicated form of HSP. In addition to sNfL and sGFAP raw levels, we used age-adjusted z-scores to account for age-related biomarker level increases.</p><p><strong>Results: </strong>In our cohort of 18 PLS patients, 18 AMN patients, 25 SPG4 patients and 60 controls, sNfL z-scores were higher in PLS than in SPG4 (<i>p</i> < 0.001), AMN (<i>p</i> = 0.03), and controls (<i>p</i> < 0.001). Furthermore, sNfL z-scores allowed distinguishing PLS from SPG4 (AUC 0.82, 95% CI 0.67-0.98) and-slightly less accurate-from AMN (AUC 0.77, 95% CI 0.60-0.95). sGFAP z-scores did not differ significantly between groups.</p><p><strong>Conclusions: </strong>Our study suggests that serum NfL, but not GFAP, is a potential diagnostic biomarker in degenerative UMN diseases and may help to differentiate PLS from pure and complicated forms of HSP. Our results indicate that axonal degeneration-the source of NfL release-is predominant over astrocytic pathology-the source of GFAP release-in PLS, AMN, and SPG4.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-8"},"PeriodicalIF":2.8,"publicationDate":"2025-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145082511","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":"Examining IGFBP7 as a potential therapeutic target in people with ALS.","authors":"Andrew Brown, Colin Stubberfield, Fernando Vieira, Richard Bedlack","doi":"10.1080/21678421.2025.2559441","DOIUrl":"https://doi.org/10.1080/21678421.2025.2559441","url":null,"abstract":"<p><p>A single nucleotide variant in an insulin-like growth factor (IGFBP7) promotor, which reduces IGFBP7 levels in brain, was previously associated with an ALS \"reversal\" phenotype. This raises the question of whether IGFBP7 might be a therapeutic target in ALS. Here, we use a combinatorial analysis to show that IGFBP7-Antisense (AS1) is associated with resistance to ALS. In ALS patients' blood, we demonstrate increased IGFPB7 protein relative to healthy controls. In ALS patients' spinal cords and iPS-derived motor neurons, we demonstrate increased IGFBP7 mRNA levels relative to healthy controls. These four new analyses support IGFBP7 as a possible therapeutic target in ALS.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-4"},"PeriodicalIF":2.8,"publicationDate":"2025-09-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145055387","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":"Dextromethorphan/quinidine (DMQ) for reducing bulbar symptoms in amyotrophic lateral sclerosis - assessment of treatment experience in a multicenter study.","authors":"Susanne Spittel, Torsten Grehl, Patrick Weydt, Dagmar Kettemann, Rachel Fabian, Annekathrin Rödiger, Uta Smesny, Robert Steinbach, Benjamin Ilse, Ute Weyen, Susanne Petri, Rea Lumi, Bogdan Bjelica, Paul Lingor, Julian Grosskreutz, Bettina M Göricke, Waltraud Pfeilschifter, Wibke Schmeja, Johannes Dorst, Alexander Mensch, Jürgen Siebert, Jenny Norden, Sarah Bernsen, Senthil Kumar Subramanian, Barbara Hildebrandt, Bertram Walter, Christoph Münch, André Maier, Thomas Meyer","doi":"10.1080/21678421.2025.2557932","DOIUrl":"https://doi.org/10.1080/21678421.2025.2557932","url":null,"abstract":"<p><strong>Background: </strong>In amyotrophic lateral sclerosis (ALS), dextromethorphan/quinidine (DMQ) has been reported to reduce bulbar symptoms, including dysarthria and dysphagia. However, data on patients' perceptions of DMQ treatment are limited.</p><p><strong>Methods: </strong>Data on DMQ treatment were collected from 1065 ALS patients treated at 13 ALS centers between 10-2015 and 06-2025. Patient-reported outcome measures (PROM) of 179 participants were remotely assessed via the \"ALS App\". PROM included the self-explanatory version of the ALS Functional Rating Scale (ALSFRS-R-SE), the Net Promoter Score (NPS); and Treatment Satisfaction Questionnaire for Medication (TSQM-9).</p><p><strong>Results: </strong>Mean disease duration was 29.3 months (SD 38.1). ALS progression before treatment was 0.82 points/month (ALSFRS-R). Mean DMQ treatment duration was 8.4 months (SD 10.8), including 35.2% (<i>n</i> = 374) of shorter (<3 months), 35.3% (<i>n</i> = 375) of longer (3-9 months), and 29.5% (<i>n</i> = 313) of very long DMQ treatment (>9 months). Patients' recommendation (<i>n</i> = 178) was positive (NPS: +23) with higher scores after very long DMQ treatment (NPS +37) compared to longer (NPS +15) and shorter treatment (NPS +7.5), respectively. TSQM-9 scores (<i>n</i> = 163) demonstrated high satisfaction for effectiveness 60.0 (SD 25.9), convenience 73.8 (SD 18.2), and global satisfaction 63.4 (SD 29.8).</p><p><strong>Interpretation: </strong>The positive perception in PROM underscores the value of DMQ as an individualized treatment option for bulbar symptoms in ALS. However, shortage of clinical data, online assessment, and selection biases are among the limitations of this study that need to be addressed in further investigations.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-13"},"PeriodicalIF":2.8,"publicationDate":"2025-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145034731","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":"Neck flexion weakness predicts respiratory dysfunction in amyotrophic lateral sclerosis.","authors":"Nathan Pavey, Alyssa Tieppo, Aicee Dawn Calma, Andrew Hannaford, Emma Grey, Bronwen Orden, Julie Ryder, Eunji Lee, Iryna Zablotska-Manos, Matthew Silsby, Parvathi Menon, Steve Vucic","doi":"10.1080/21678421.2025.2557939","DOIUrl":"https://doi.org/10.1080/21678421.2025.2557939","url":null,"abstract":"<p><p><i>Objective</i>: Neck flexion (NF) weakness is a frequently observed clinical feature in amyotrophic lateral sclerosis (ALS), particularly in advanced disease. The aim of the present study was to assess whether NF weakness could be a clinical biomarker for development of respiratory dysfunction. <i>Methods</i>: Sixty-two ALS patients were prospectively recruited at Brain and Nerve Research Center. Neck flexion strength was assessed by the Medical Research Council (MRC) score and handheld dynamometry (HHD). Respiratory function testing was assessed by spirometry, including forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1). Site of disease onset, disease duration, and ALSFRS-R were recorded. <i>Results</i>: Neck flexion weakness (MRC ≤4) was evident in 27% of ALS patients. There was a significant reduction of FVC in ALS patients with weak NF (ALS_NFweakness 70.0 ± 7.2%; ALS_NFnormal 86.8 ± 2.4% predicted, <i>p</i> = 0.038). Additionally, reduction of HHD measurements was significantly correlated with FVC (<i>R</i> = 0.487, <i>p</i> < 0.001) and FEV1 (<i>R</i> = 0.465, <i>p</i> < 0.001), and was most prominent in bulbar onset ALS (FVC: <i>R</i>² = 0.673, <i>p</i> = 0.002). Of relevance, the presence of NF weakness (MRC ≤ 4) was a significant predictor of reduced FVC ≤50% predicted (Chi² = 7.68, <i>p</i> = 0.006), a threshold indicating need for ventilatory support. <i>Conclusion</i>: Neck flexion weakness, particularly when quantified by the MRC score and HHD, serves as a marker of respiratory dysfunction in ALS patients. This simple clinical assessment may herald the development of respiratory dysfunction and requirement for respiratory ventilatory support.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-7"},"PeriodicalIF":2.8,"publicationDate":"2025-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145034764","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":"What is the influence of fatigue, ALSFRS-R scores, cognitive status, and pain in individuals with Amyotrophic Lateral Sclerosis? A cross-sectional study.","authors":"Stephano Silva, Diogo Aires, Aline Souza, João Macedo, Luciana Melo, Saionara Câmara, Ricardo Valentim, Ana Raquel Lindquist, Giane Samora, Tatiana Ribeiro","doi":"10.1080/21678421.2025.2557971","DOIUrl":"https://doi.org/10.1080/21678421.2025.2557971","url":null,"abstract":"<p><p><i>Introduction:</i> Fatigue remains a poorly understood symptom in individuals with ALS, and little is known about its associtation with other symptoms, including functional impairment, cognition, and pain. <i>Objective:</i> To identify the levels of fatigue, pain, ALSFRS-R, and cognition of a Brazilian group of individuals with ALS, in order to verify possible influences between these symptoms and fatigue. <i>Methods:</i> This is a cross-sectional study conducted with individuals with ALS who were recruited intentionally, using a non-probabilistic sampling method. After agreeing to participate, they were assessed using a standardized assessment form, and data regarding fatigue level, ALSFRS-R scores, cognition, and pain were collected. Data were analyzed by categorizing fatigue (with and without fatigue) and considering sociodemographic and clinical covariates, followed by comparisons, bivariate analyses, and multiple linear regression analyses. <i>Results:</i> Data were collected from 72 individuals with ALS. Inferential statistics indicated differences between fatigue categories concerning ALSFRS-R scores, cognition, and pain. After multiple linear regression analyses, an association between fatigue and the dependent variables was identified. <i>Conclusion:</i> Fatigue is associated with lower ALSFRS-R scores, poorer cognitive status, and higher levels of pain in a Brazilian cohort of ALS. Additionally, age, sex, education, and length of illness were identified as potential factors for fatigue occurrence, observed more frequently in females than in males with the condition.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-8"},"PeriodicalIF":2.8,"publicationDate":"2025-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145031223","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":"Reliable change indices for the cognitive section of Portuguese version of the Edinburgh Cognitive and Behavioural ALS screen (ECAS).","authors":"Inês Alves, Edoardo Nicolò Aiello, Diana Lopes, Marta Gromicho, Sara Simão, Arianna Moreschi, Giulia De Luca, Beatrice Curti, Vincenzo Silani, Nicola Ticozzi, Barbara Poletti, Mamede de Carvalho","doi":"10.1080/21678421.2025.2555216","DOIUrl":"10.1080/21678421.2025.2555216","url":null,"abstract":"<p><p>This study aimed to derive standardized regression-based (SRB) reliable change indices (RCIs) for the cognitive section of the Portuguese Edinburgh Cognitive and Behavioral ALS Screen (ECAS-C). Forty-nine MND patients undergoing the ECAS were followed-up (T1) at 7.2 ± 2 months (range = 5-12). RCIs were derived <i>via</i> an SRB approach by accounting for demographic, motor-functional and test-related variables. Practice effects were detected as to Total and Memory measures; all ECAS-C measures proved to be reliable at retest. Baseline ECAS-C measures predicted their follow-up performances. SRB RCIs herewith delivered will help assess MND patients' cognition over time, although they would benefit from further validation in independent cohorts.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-4"},"PeriodicalIF":2.8,"publicationDate":"2025-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145008637","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}