Amyotrophic lateral sclerosis & frontotemporal degeneration最新文献

{"title":"Epidemiology of amyotrophic lateral sclerosis: a population-based analysis, 2015-2020.","authors":"Bernat Bertran-Recasens, Sergio Vidal-Notari, Guillem Hernández Guillamet, Francesc López Seguí, Josep Vidal-Alaball, Joan Jiménez-Balado, Miguel Angel Rubio","doi":"10.1080/21678421.2025.2527887","DOIUrl":"10.1080/21678421.2025.2527887","url":null,"abstract":"<p><p><i>Background:</i> Epidemiological data on amyotrophic lateral sclerosis (ALS) in Spain have primarily been derived from small cohort studies, with limited information on survival and comorbidities. This study presents a 10-year follow-up of a large, well-phenotyped community-dwelling ALS cohort in Catalonia, Spain. <i>Methods:</i> This observational study utilized data from the Information System for the Development of Research in Primary Care (SIDIAP), which includes health records for 6,301,095 individuals from 2015 to 2020. We assessed ALS incidence, prevalence, comorbidities, territorial distribution, mortality, and survival times. <i>Results:</i> From 2015 to 2020, 1173 ALS cases were identified, with a median age at diagnosis of 68 years, and 50.4% of cases were female. Incidence and prevalence were estimated at 2.39 per 100,000 person-years and 7.98 cases per 100,000 persons. Dementia was present in 6.8% of cases before ALS diagnosis, while depression and/or anxiety affected 45.7%. Median survival from diagnosis was 2.19 years. Multivariate analysis identified older age at diagnosis (HR: 1.04, 95% CI: 1.04-1.05, <i>p</i> value < 0.001), alcohol abuse (HR: 1.56, 95% CI: 1.04-2.56, <i>p</i> value = 0.017), history of stroke (HR: 1.47, 95% CI: 1.07-2.04, <i>p</i> = 0.006), and dementia (HR: 1.57, 95% CI: 1.18-2.12, <i>p</i> value = 0.001) as independent predictors of mortality. <i>Conclusions:</i> ALS incidence and prevalence in Catalonia are higher than previously estimated for Spain and align closely with rates observed in other Western countries. Older age at diagnosis, alcohol abuse, stroke history, and dementia were all significantly associated with reduced survival. These findings underscore important risk factors affecting prognosis, offering valuable insights into ALS progression.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"784-793"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144612358","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":"Neuropsychological assessment practices in PRECISION-ALS: challenges and opportunities for harmonization.","authors":"Emmet Costello, Joke De Vocht, Emily Beswick, Éanna Mac Domhnaill, Colm Peelo, Juliette Foucher, Emily J Mayberry, Theresa Chiwera, Fenna Hiemstra, Alejandro Caravaca Puchades, Barbara Iazzolino, Francesca Palumbo, Inês Alves, Elisabeth Kasper, Miriam Galvin, Mark Heverin, Caroline Ingre, Christopher J Mcdermott, Pamela Shaw, Ammar Al-Chalabi, Leonard H Van Den Berg, Mónica Povedano Panadés, Adriano Chiò, Mamede De Carvalho, Sofiane Bencheikh, Philippe Corcia, Mohammed Mouzouri, Andreas Hermann, Sharon Abrahams, Niall Pender, Philip Van Damme, Orla Hardiman","doi":"10.1080/21678421.2025.2527877","DOIUrl":"10.1080/21678421.2025.2527877","url":null,"abstract":"<p><strong>Objective: </strong>To gather comprehensive insights regarding current neuropsychological assessment practices in PRECISION-ALS, a pan-European research and industry consortium, to propose areas which can be harmonized and facilitate more robust cross-country comparisons.</p><p><strong>Methods: </strong>Representatives from PRECISION-ALS sites were surveyed with a semi-structured interview, gathering information on how people with ALS are assessed for cognitive/behavioral change, including how they are initially screened, classified as impaired/unimpaired, and followed up longitudinally. Assessment practices across PRECISION-ALS sites were summarized using descriptive analysis.</p><p><strong>Results: </strong>Ten of the eleven PRECISION-ALS sites perform cognitive and/or behavioral screening at least once during the course of the disease, using the Edinburgh Cognitive and Behavioral ALS Screen, either for clinical or research purposes. All centers categorize impairment, but differ how it is defined, with some using local norms, and others using other countries' norms. Most sites account for age and education, but differ in how these factors are considered. Longitudinal protocols vary in terms of the number of assessments, time intervals, and use of alternative versions. Behavioral screening is more consistently implemented, with the ECAS caregiver interview as the standard tool, however there is a lack of clarity in how this data is applied. Many sites supplement cognitive and behavioral screening with additional measures of mood and/or neuropsychiatric symptoms.</p><p><strong>Conclusions: </strong>These findings illustrate areas of commonality and divergence in neuropsychological screening practices. Site-specific variations are likely to confound research involving cross-country data-sharing. PRECISION-ALS, in generating prospective population-based datasets, will provide agreed harmonized protocols for neuropsychological assessment across participating sites.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"748-757"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676656","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":"Understanding the uptake and use of noninvasive ventilation among Australians living with amyotrophic lateral sclerosis: results of a national survey.","authors":"Marnie Graco, Kate A Carey, Kylie Russo, Nicole L Sheers, David J Berlowitz","doi":"10.1080/21678421.2025.2529406","DOIUrl":"https://doi.org/10.1080/21678421.2025.2529406","url":null,"abstract":"<p><p><i>Objective:</i> Noninvasive ventilation (NIV) improves quality of life and extends survival in amyotrophic lateral sclerosis (ALS), yet NIV uptake among Australians with ALS has been estimated at 19%. This study aimed to identify demographic and disease-related factors associated with NIV uptake among people with ALS (pwALS). <i>Methods:</i> A national cross-sectional survey. PwALS (or their family caregivers) completed an online survey about their NIV use and healthcare experiences. Survey data were analyzed descriptively. Associations between demographic factors and three dichotomous NIV outcomes: \"using NIV\"; \"offered and accepted NIV\"; and \"discussed NIV with a healthcare professional (HCP)\" were investigated using multivariate logistic regression modeling. <i>Results:</i> A total of 224 responses were received, of which 201 completed the demographic questions. Mean (SD) age was 64 (11) years, 62% were male, and median (IQR) time since diagnosis was 2 (1-5) years. Forty-six percent were using NIV; 6% had started NIV and stopped; 4% had accepted a referral but not started; 3% had declined NIV; and 26% had never discussed NIV with a HCP. Demographic factors positively associated (<i>p</i> < 0.05) with at least one NIV outcome included: being male, age < 65 years, residing in a metropolitan/regional area, attending a ALS multidisciplinary clinic, and longer time since diagnosis. <i>Conclusion:</i> NIV uptake among Australians with ALS appears to have increased in the last decade, however this survey identified concerning disparities related to sex, age, and location of residence. Research exploring the underlying causes of these disparities is urgently required so that targeted interventions can be designed and implemented.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":"26 7-8","pages":"649-658"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145310140","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":"Incidence of ALS in all 50 states in the United States, data from the National ALS Registry, 2012-2019.","authors":"Paul Mehta, Jaime Raymond, Theresa Nair, Moon Han, Reshma Punjani, Theodore Larson, Jasmine Berry, Suraya Mohidul, Song Xue, D Kevin Horton","doi":"10.1080/21678421.2025.2506448","DOIUrl":"10.1080/21678421.2025.2506448","url":null,"abstract":"<p><strong>Objective: </strong>To summarize amyotrophic lateral sclerosis (ALS) incidence in all 50 states and the District of Columbia from 2012 to 2019. In 2010, the Agency for Toxic Substances and Disease Registry (ATSDR) launched the congressionally mandated National ALS Registry (Registry) to determine the incidence and prevalence of ALS within the United States, characterize demographics, and identify potential risk factors. This is the first analysis of state-level ALS incidence estimates for all 50 states and the District of Columbia.</p><p><strong>Methods: </strong>ALS is not a notifiable disease in the United States. As such, the Registry identifies cases using existing national administrative databases (Medicare, Veterans Health Administration, and Veterans Benefits Administration), and a secure web portal that identifies cases not included in the national databases. Confirmed and likely cases are deduplicated and counted as incident cases for the first year they are identified using a validated algorithm. Incident counts, incident rates, and rate ratios were then calculated.</p><p><strong>Results: </strong>State-level age-adjusted overall incidence rates for 2012 to 2019 ranged from 0.65 per 100,000 persons (Alaska) to 2.25 per 100,000 persons (Vermont), with an overall incidence of 1.44 per 100,000 persons in the United States. New England and the upper Midwest regions had higher incidence rates than national rates.</p><p><strong>Conclusions: </strong>These findings summarize the incidence of ALS for all 50 states from 2012 to 2019. This is a continuing effort to identify ALS cases nationally. The establishment of the Registry allows for epidemiological analyses of ALS data and the assessment of potential risk factors.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"758-766"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144200906","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":"Hospitalizations as an outcome measure in COURAGE-ALS.","authors":"Stacy A Rudnicki, Ammar Al-Chalabi, Jinsy A Andrews, Adriano Chio, Philippe Corcia, Philippe Couratier, Merit E Cudkowicz, Mamede De Carvalho, Angela Genge, Orla Hardiman, Terry Heiman-Patterson, Robert D Henderson, Caroline Ingre, Wendy Johnston, Albert Ludolph, Nicholas J Maragakis, Timothy M Miller, Jesus S Mora, Susanne Petri, Zachary Simmons, Leonard H Van Den Berg, Lorne Zinman, Katherine E Herder, Stuart Kupfer, Fady I Malik, Lisa Meng, Tyrell J Simkins, Jenny Wei, Andrew A Wolff, Jeremy M Shefner","doi":"10.1080/21678421.2025.2515907","DOIUrl":"10.1080/21678421.2025.2515907","url":null,"abstract":"<p><p><i>Objective</i>: To describe the development of a methodology to characterize hospitalizations and their relationship to amyotrophic lateral sclerosis (ALS) and provide results using this process in a phase 3 trial of <i>reldesemtiv</i> in ALS. <i>Methods</i>: ALS clinical trialists assisted in developing a classification system to determine if a hospitalization was related to ALS (HR-ALS), unrelated (HU-ALS), or if the relationship was indeterminate (HI-ALS) and this was applied by the investigators to hospitalizations in COURAGE-ALS. Time to first hospitalization and number of hospitalizations were compared between those assigned <i>reldesemtiv</i> or placebo for up to 48 weeks. Demographic and clinical features were evaluated for prediction of hospitalization risk; this analysis was limited to those participants who completed the first 24-week double-blind placebo-controlled portion of the trial. <i>Results</i>: COURAGE-ALS terminated early due to futility. Time to first hospitalization was similar in the <i>reldesemtiv</i> compared to placebo arms as was the incidence, with 86 of the participants (17.6% of those originally assigned placebo and 18.0% originally on <i>reldesemtiv</i>) experiencing an event. The largest percentage of events was classified as HR-ALS for both placebo (64%, 18/28) and <i>reldesemtiv</i> (76%, 44/58). In a multivariate model, only bulbar or respiratory onset disease was a significant risk factor for hospitalization. <i>Conclusion</i>: While most hospitalizations in COURAGE-ALS were HR-ALS, HU-ALS and HI-ALS also occurred. When using hospitalization as an endpoint in an ALS clinical trial, recording its relationship to ALS provides additional details to characterize disease burden and clinical meaningfulness of the endpoint.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"802-811"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144276907","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":"Symptoms prior to diagnosis among a diverse patient population with amyotrophic lateral sclerosis In the USA.","authors":"Joseph Guilfoyle, Yunhua Fan, Olga SÁnchez-SoliÑo, Joey Boiser, Lisa Vinikoor-Imler","doi":"10.1080/21678421.2025.2538599","DOIUrl":"10.1080/21678421.2025.2538599","url":null,"abstract":"<p><strong>Background: </strong>Amyotrophic lateral sclerosis (ALS) symptom onset is gradual and may include muscle weakness, dysarthria, dysphagia, and respiratory difficulties among others. The objective of this study is to describe sex and racial/ethnic variation in limb and bulbar symptom diagnoses before ALS diagnosis in the USA.</p><p><strong>Methods: </strong>This retrospective cohort study was conducted using Optum's de-identified Market Clarity Data with a patient identification period between January 2015 and December 2019. Cases were identified using ICD-9/10 codes and were required to have ≥3 years of continuous enrollment or EHR activity prior to diagnosis. Descriptive statistics of symptom frequency and onset were stratified by sex and race.</p><p><strong>Results: </strong>This study identified 7121 individuals with ALS, 3303 female (46%) and 3818 male (54%). Most identified as Non-Hispanic White (67.5%), followed by African American (6.6%), Hispanic (3.6%), and Asian (0.9%), with 21.4% missing race/ethnicity. In the 3 years before ALS diagnosis, 42.9% of subjects were diagnosed with ≥1 bulbar symptom, 74.5% with ≥1 limb symptom, and 34.6% with both. Females more likely to be diagnosed than males: any bulbar 47.1% versus 39.3%, (<i>p</i> < 0.0001), any limb 76.0% versus 73.2%, (<i>p</i> = 0.007), both 38.1% versus 31.6%, (<i>p</i> < 0.0001). Variation by race/ethnicity was observed for limb symptoms (<i>p</i> < 0.0001) and both bulbar and limb symptoms (<i>p</i> = 0.008), but not bulbar symptoms alone (<i>p</i> = 0.07). Symptom onset to ALS was longer for females and varied by race/ethnicity.</p><p><strong>Conclusion: </strong>Females were more likely to present with bulbar and limb symptoms prior to ALS diagnosis and African American patients were more likely to present with limb symptoms than other race/ethnicities.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"664-672"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144762466","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 cognitive decline over time in Iranian ALS patients: Adapting successive versions B and C of the Edinburgh cognitive and behavioral screen to Persian.","authors":"Zoha Shaka, Helia Mojtabavi, Morvarid Taebi, Behrooz Mahmoodi-Bakhtiari, Payam Sarraf","doi":"10.1080/21678421.2025.2509615","DOIUrl":"10.1080/21678421.2025.2509615","url":null,"abstract":"<p><strong>Objective: </strong>To adapt successive versions B and C of the Edinburgh Cognitive and Behavioral Screen (ECAS) into Persian and evaluate cognitive and behavioral changes over time in Iranian ALS patients.</p><p><strong>Methods: </strong>This study included 38 ALS patients in the ECAS-B group and 29 in the ECAS-C group, diagnosed between May 2021 and February 2023 at the Iranian Center of Neurological Research, Imam Khomeini Hospital, Tehran, Iran. Additionally, 59 age- and education-matched healthy controls were enrolled (30 for ECAS-B and 29 for ECAS-C). The Montreal Cognitive Assessment (MoCA) was used to validate the ECAS versions.</p><p><strong>Results: </strong>The Persian versions of ECAS-B and ECAS-C demonstrated strong internal consistency (Cronbach's alpha: 0.88 for ECAS-B and 0.89 for ECAS-C) and a positive correlation with MoCA and ALS-FRS-r scores. The area under the ROC curve was 0.851 for ECAS-B and 0.861 for ECAS-C. ECAS-C scores were significantly lower than ECAS-B scores, suggesting a faster cognitive decline over time. Optimal cutoff values of 72 for ECAS-B and 78 for ECAS-C were identified for detecting cognitive impairment. Cognitive impairment was observed in 10 patients (26.31%) in the ECAS-B group and 15 patients (51.72%) in the ECAS-C group.</p><p><strong>Conclusions: </strong>The Persian versions of ECAS-B and ECAS-C demonstrate good validity and reliability for detecting cognitive impairment and tracking cognitive decline in ALS patients.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"709-719"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144217652","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":"Educational adjustments for the Chinese version of the Edinburgh Cognitive and Behavioral Screen (ECAS): establishing normative data.","authors":"Min Wang, Xiaoxian Guo, Hua Zhang, Nan Zhang, Tielun Yin, Yan Gao, Jing Kang, Yanying Hu, Dongsheng Fan, Shan Ye","doi":"10.1080/21678421.2025.2539904","DOIUrl":"10.1080/21678421.2025.2539904","url":null,"abstract":"<p><strong>Background: </strong>The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) is a cognitive screening tool designed for patients with amyotrophic lateral sclerosis (ALS). It has been translated into various languages and used globally. This study aimed to establish normative data for the Chinese version of the ECAS to better serve the Mandarin population.</p><p><strong>Methods: </strong>We enrolled 358 healthy individuals from different regions of China, all of whom completed the ECAS, and 340 also completed the Mini-Mental State Examination (MMSE). The participants were categorized into six age groups and five education groups.</p><p><strong>Results: </strong>ECAS scores were found to be related to education level (<i>p</i> < 0.001). After Bonferroni correction for multiple comparisons, we determined that there were no significant differences in total ECAS scores between the junior middle school and senior middle school groups, leading to their combination into a single middle school group. We established cutoff values for the ECAS on the basis of education group. After adjusting for age, sex, and education level, a significant correlation was found between MMSE scores and total ECAS scores (<i>p</i> < 0.001), indicating a high level of consistency in the cognitive assessment.</p><p><strong>Conclusion: </strong>We have established norms for the ECAS on the basis of education level, and the ECAS is highly consistent with the MMSE in the assessment of cognition. These norms will be instrumental in future clinical and follow-up work for Mandarin ALS patients.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"702-708"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144735809","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":"Federated sport activity in amyotrophic lateral sclerosis: a case-control study.","authors":"Inês Alves, Marta Gromicho, Ana Catarina Pronto-Laborinho, Diana Lopes, Miguel Oliveira Santos, Mamede De Carvalho","doi":"10.1080/21678421.2025.2511124","DOIUrl":"10.1080/21678421.2025.2511124","url":null,"abstract":"<p><p>Amyotrophic lateral sclerosis (ALS) develops in a multistep process combining environmental variables and genes. Among the identified risk factors, the role of regular vigorous physical activity is still debatable. <i>Objective:</i> This case-control study investigated the relationship between ALS and different degrees of sports engagement, with federated status as a proxy for strenuous activity. <i>Methods:</i> 586 ALS patients and 558 controls were consecutively assessed by using a standard questionnaire. Due to low female participation in regular or intensive sports activity, the study focused on men (327 with ALS and 314 controls). <i>Results:</i> Overall, football (soccer) had the most practitioners (n = 137, 35.8%), accounting for 62.1% of ALS and 32.3% of control federated athletes. Male football players have a 3.07-fold increased ALS risk (95% CI: 1.82-5.19) compared to other men (<i>p</i> < 0.0001) and 3.43-fold increase (95% CI: 1.77-6.68) compared to those federated in other sports (<i>p</i> = 0.0003). After controlling for age and trauma, football players still had 2.91-fold (95% CI: 1.70-5.01) increased risk compared to non-federated and non-participants in contact sports intensively. No significant ALS risk difference existed for other sports practiced with identical intensity and contact levels. Clinical characteristics of ALS federated football players were similar to other ALS patients. <i>Conclusion:</i> Our results suggest ALS susceptibility is not linked to general physical activity, but specifically to competitive football, regardless of a history of head and neck trauma. Given football's popularity, even a small risk increase could impact many. Further research is required to understand the mechanisms linking football to ALS, and why this association is not observed in other sports.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"767-774"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144251073","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":"Variability across versions of the self-administered ALSFRS-R: a review and call for harmonization.","authors":"Matti D Allen, Ruben P A Van Eijk, Liam Knox, Jill Carlton, Esther Hobson, Christopher J Mcdermott, Deirdre Murray, James Berry, Thomas Meyer, Angela Genge","doi":"10.1080/21678421.2025.2522400","DOIUrl":"10.1080/21678421.2025.2522400","url":null,"abstract":"<p><p>Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease predominantly affecting motor neurons resulting in substantial, progressive disability. The amyotrophic lateral sclerosis functional rating scale - revised (ALSFRS-R) is commonly used to assess and monitor functional status in patients with ALS. Additionally, it is the current regulatory accepted primary outcome measure documenting functional status in ALS clinical trials. The ALSFRS-R was originally designed to be administered to a patient by a trained professional. But over time it has been adapted to be performed independently by patients or their caregivers without assistance. Several different versions of the self-administered ALSFRS-R have been created over the past two decades, each with subtle but important differences. Some of these differences are related to language used in item wording or the platform for which the scale was intended to be administered (e.g. digitally). These differences across versions of the self-administered scale may be problematic as they could increase the heterogeneity of data collected across clinical trials or complicate interpretation of results across trials. Therefore, we highlight the need for a harmonized version of the self-administered ALSFRS-R to be used across all clinics and clinical trial sites internationally.</p>","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"631-636"},"PeriodicalIF":2.8,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144531359","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}