Amyotrophic lateral sclerosis & frontotemporal degeneration最新文献_第7页

Another family with ALS and homozygosity for p.Val120Leu (c.358G > C) mutation of SOD1. 另一个ALS家族，SOD1的p.Val120Leu （C . 358g > C）突变纯合子。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-08-01 Epub Date: 2025-01-31 DOI: 10.1080/21678421.2025.2457973

Faa Gondim, José Marcelino Aragão Fernandes

引用次数: 0

Impact of relative deprivation and ethnicity on the incidence rate of amyotrophic lateral sclerosis. 相对贫困和种族对肌萎缩侧索硬化症发病率的影响。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-08-01 Epub Date: 2025-02-19 DOI: 10.1080/21678421.2025.2465609

James Alder, Chukwu Chukwuma, Tracey Farragher, Samantha Holden Smith, Rosemary Morris, John Ealing, Hisham Hamdalla, Andrew Bentley, Saba Bokhari, Debbie Freeman, Ammar Al-Chalabi, David Rog, Joyutpal Das, Amina Chaouch

{"title":"Impact of relative deprivation and ethnicity on the incidence rate of amyotrophic lateral sclerosis.","authors":"James Alder, Chukwu Chukwuma, Tracey Farragher, Samantha Holden Smith, Rosemary Morris, John Ealing, Hisham Hamdalla, Andrew Bentley, Saba Bokhari, Debbie Freeman, Ammar Al-Chalabi, David Rog, Joyutpal Das, Amina Chaouch","doi":"10.1080/21678421.2025.2465609","DOIUrl":"10.1080/21678421.2025.2465609","url":null,"abstract":"Objective: This study assessed a sizable cohort of patients with amyotrophic lateral sclerosis (ALS) in a relatively deprived and ethnically diverse area in the northwest of England. We aimed to evaluate the interaction of relative deprivation and ethnicity with the incidence of ALS. Methods: Six hundred and ninety-three adults from Greater Manchester who were diagnosed with ALS between 1 January 2011 and 31 December 2021 were included in this study. Data were collected from electronic patient records. Relative deprivation was estimated using the Index of Multiple Deprivation 2019 and patients were divided into quartiles of deprivation in England. Ethnicity was sub-grouped into White, Southeast Asian, Black, and Other. Poisson's regression analysis was used to calculate the incidence rate and its interactions with deprivation and ethnicity. Results: 55.4% of patients were male, 95.4% were White, 57.4% were in the two most deprived quartiles, and 87.2% had died by the end of the observation period. The crude incidence rate was 2.21 cases per 100,000 (95% CI 2.00-2.40) per year. There was no difference in the adjusted incidence rates among the quartiles of deprivation, even when considering ethnicity as a confounding variable. The risk of ALS in the White population was 2.08 (95% CI 1.47-3.04) times greater than that in the non-White population. Conclusion: In our cohort, relative deprivation was not an independent risk factor for ALS. A stronger association between White ethnicity and ALS was noted. The reason for this association remains unclear, highlighting the need for more research in this field.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"558-565"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143451021","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}

引用次数: 0

Nutritional care practices in ALS: perspectives of healthcare professionals and people with ALS. 肌萎缩侧索硬化症的营养护理实践：保健专业人员和肌萎缩侧索硬化症患者的观点。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-08-01 Epub Date: 2025-05-15 DOI: 10.1080/21678421.2025.2501681

Merle M Kuiper, Willeke J Kruithof, Nicole Broekman-Peters, Dea L Schröder-van den Nieuwendijk, Johanna M A Visser-Meily, Anita Beelen

{"title":"Nutritional care practices in ALS: perspectives of healthcare professionals and people with ALS.","authors":"Merle M Kuiper, Willeke J Kruithof, Nicole Broekman-Peters, Dea L Schröder-van den Nieuwendijk, Johanna M A Visser-Meily, Anita Beelen","doi":"10.1080/21678421.2025.2501681","DOIUrl":"10.1080/21678421.2025.2501681","url":null,"abstract":"Objective: To map nutritional care provided to people with ALS, PMA and PLS (pwALS) and identify barriers encountered by healthcare professionals and pwALS. Methods: Two online questionnaires, addressing current nutritional management of ALS in the Netherlands, were sent to healthcare professionals of the 36 certified ALS care teams and pwALS drawn from a population-based registry. Topics were: 1) contact between pwALS and their ALS care team, 2) monitoring nutritional status, 3) nutritional advice provided or received, and 4) satisfaction with current nutritional care and barriers encountered. Results: In total, 100 healthcare professionals and 372 pwALS completed the questionnaires. Dietitian responses (n = 36/100) showed that 28% utilized malnutrition screening tools and 17% measured body composition. Dietitians used different predictive equations to estimate energy and protein requirements. Patient responses showed that 50% had contact with a dietitian, 7% indicated body composition had been measured and 25% reported never being weighed or weighing themselves. Healthcare professionals and pwALS highlighted the need for comprehensive, up-to-date information on nutrition and ALS, national consensus on nutritional advice and monitoring methods, patient information material, training for healthcare professionals and personalized nutritional advice for pwALS. Conclusions: Practice variation was observed in the assessment and monitoring of nutritional status and the provision of nutritional advice. Suboptimal monitoring of nutritional status and estimation of nutritional requirements may result in delayed detection of malnutrition and inaccurate dietary recommendations. Further research and national consensus on monitoring methods and nutritional advice is required.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"452-466"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144082484","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}

引用次数: 0

Patients' choices regarding ventilatory support affect opioid use in amyotrophic lateral sclerosis. 肌萎缩性侧索硬化症患者对通气支持的选择影响阿片类药物的使用。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-08-01 Epub Date: 2025-01-24 DOI: 10.1080/21678421.2025.2453463

Chiharu Matsuda, Yuki Nakayama, Michiko Haraguchi, Ryo Morishima, Yumi Itagaki, Kota Bokuda, Hideki Kimura, Kazushi Takahashi, Toshio Shimizu

{"title":"Patients' choices regarding ventilatory support affect opioid use in amyotrophic lateral sclerosis.","authors":"Chiharu Matsuda, Yuki Nakayama, Michiko Haraguchi, Ryo Morishima, Yumi Itagaki, Kota Bokuda, Hideki Kimura, Kazushi Takahashi, Toshio Shimizu","doi":"10.1080/21678421.2025.2453463","DOIUrl":"10.1080/21678421.2025.2453463","url":null,"abstract":"Objective: To investigate the impact of different ventilatory support options on opioid use among patients with amyotrophic lateral sclerosis (ALS).Methods: We retrospectively reviewed 889 consecutive patients with ALS and enrolled 399 eligible patients. All patients were followed until death or tracheostomy. Clinical characteristics of patients and the timing of initial opioid administration were evaluated. Patients were categorized into four subgroups: (1) 160 patients who never used a ventilator, (2) 120 patients who used only noninvasive ventilation (NIV), (3) 61 patients who transitioned from NIV to tracheostomy and invasive ventilation (TIV), and (4) 58 patients who underwent TIV without prior NIV. We compared the prevalence of opioid use across these groups and assessed its relationship with ventilatory support options using multivariate logistic analysis.Results: A total of 130 patients (32.6%) used opioids. The number of patients who used opioids in each group was as follows: 55 (34.4%) in Group 1, 69 (57.5%) in Group 2, 5 (8.2%) in Group 3, and 1 (1.7%) in Group 4 (p < 0.0001). Multivariate logistic analysis revealed that, compared to Group 1, the use of NIV only was positively associated with opioid use (p = 0.002). In contrast, transitioning from NIV to TIV (Group 3) and using TIV only (Group 4) were negatively associated with opioid use (p = 0.0001 and 0.001, respectively).Conclusions: The choice of ventilatory support significantly influences opioid use in patients with ALS. Patients who opted against TIV required opioids to relieve distress more commonly than those who chose TIV.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"409-416"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143029889","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}

引用次数: 0

Epidemiology and healthcare utilization in motor neuron disease in Czechia. 捷克运动神经元病的流行病学及保健利用。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-08-01 Epub Date: 2025-05-21 DOI: 10.1080/21678421.2025.2506441

Nazli Sila Kara, Anna Zouharova, Hana Marie Broulikova, Jiri Jarkovsky, Lenka Slachtova

{"title":"Epidemiology and healthcare utilization in motor neuron disease in Czechia.","authors":"Nazli Sila Kara, Anna Zouharova, Hana Marie Broulikova, Jiri Jarkovsky, Lenka Slachtova","doi":"10.1080/21678421.2025.2506441","DOIUrl":"10.1080/21678421.2025.2506441","url":null,"abstract":"The demographic and epidemiological profile of motor neuron disease (MND) in Czechia remains unknown, highlighting a critical gap; this study aims to examine the epidemiology of MND using population-based data. We conducted a 10-year retrospective study (2012-2022) to analyze prevalence, incidence, mortality, and healthcare utilization, including Riluzole treatment and multidisciplinary care. Our epidemiological findings were compared with the Global Burden of Disease estimates to highlight the importance of real-world data. Based on the data from 4583 patients with MND, the mean annual age-standardized incidence is 3.91 (95% confidence interval [CI] 3.73 - 4.09) per 100,000 individuals, and the mean annual age-standardized prevalence is 10.43 (95% CI 9.91 - 10.95) per 100,000 individuals. The incidence and mortality rates are stable, whereas the prevalence slowly increases. A total of 58.9% of the patients are male. Median survival time is 4.27 years. Most patients, 63.7%, die in hospital, followed by home 27.9%. From healthcare services, physiotherapy and rehabilitation are the most frequently utilized, with 67% of patients benefiting. Only 6.2% of patients use genetic counseling. Riluzole is used by 43% of patients with MND. Comparing incidence and prevalence rates with Global Burden Data estimates and local studies from European countries, we identified differences highlighting the need for real-world data. This study provides crucial real-world data on MND epidemiology and healthcare consumption in Czechia, shedding light on a previously under-researched area. These insights could navigate healthcare resource use and evidence-based policies, improving outcomes for patients and caregivers while deepening understanding of MND in Europe.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"541-549"},"PeriodicalIF":0.0,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144112839","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}

引用次数: 0

Research access barriers in amyotrophic lateral sclerosis. 肌萎缩侧索硬化症的研究准入障碍。

IF 2.8

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-07-31 DOI: 10.1080/21678421.2025.2539900

Elisa Giacomelli, Erica Scirocco, Max Higgins, Arian Pilja, Sabrina Paganoni, Doreen Ho

引用次数: 0

Repurposing FDA-approved drugs for treatment of amyotrophic lateral sclerosis using machine learning. 利用机器学习重新利用fda批准的药物治疗肌萎缩侧索硬化症。

IF 2.8

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-07-31 DOI: 10.1080/21678421.2025.2536027

Saanvi Dogra, Valentina L Kouznetsova, Igor F Tsigelny

{"title":"Repurposing FDA-approved drugs for treatment of amyotrophic lateral sclerosis using machine learning.","authors":"Saanvi Dogra, Valentina L Kouznetsova, Igor F Tsigelny","doi":"10.1080/21678421.2025.2536027","DOIUrl":"https://doi.org/10.1080/21678421.2025.2536027","url":null,"abstract":"Introduction: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease characterized by loss of motor neurons. Current medications are largely ineffective, associated with side effects, and hindered by a lack of agreement over treatment pathways. The time-intensive process and high costs further limit the development of therapeutics. Therefore, this research aimed to identify FDA-approved drugs that inhibit three proteins (Casein kinase 1, Protein tyrosine kinase 2, Ephrin type-A receptor 4) associated with ALS.Methods: A machine learning (ML) model was trained for each protein to identify an inputted compound as an active inhibitor of that protein. The FDA-approved drugs were then screened through these models, and 18 drugs were identified as likely inhibitors for all three proteins. The results were validated through protein-ligand docking of each drug to its respective protein(s).Results: Risperidone was the most active drug, with an average ML score of 1 and binding affinity of -8.9. The ML scores and binding affinities had a strong correlation, indicating reliability.Conclusion: This research predicted multiple drugs that can simultaneously target many proteins involved in ALS, creating more effective treatment options at a lower cost. This procedure can be applied to efficiently discover drugs for other diseases in the future.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-9"},"PeriodicalIF":2.8,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144762465","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}

引用次数: 0

Fatigue in amyotrophic lateral sclerosis/motor neuron disease: prevalence, influences and trajectories. 肌萎缩性侧索硬化症/运动神经元疾病的疲劳：患病率、影响和轨迹。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-07-24 DOI: 10.1080/21678421.2025.2533881

Carolyn A Young, Amina Chaouch, Christopher J Mcdermott, Ammar Al-Chalabi, Suresh Kumar Chhetri, Caroline Bidder, Cathy Ellis, Joe Annadale, Roger J Mills, Alan Tennant

{"title":"Fatigue in amyotrophic lateral sclerosis/motor neuron disease: prevalence, influences and trajectories.","authors":"Carolyn A Young, Amina Chaouch, Christopher J Mcdermott, Ammar Al-Chalabi, Suresh Kumar Chhetri, Caroline Bidder, Cathy Ellis, Joe Annadale, Roger J Mills, Alan Tennant","doi":"10.1080/21678421.2025.2533881","DOIUrl":"https://doi.org/10.1080/21678421.2025.2533881","url":null,"abstract":"Objective: In a large cohort of people with amyotrophic lateral sclerosis/motor neuron disease (pwALS), we examined the age-sex prevalence of fatigue, its relationship to other symptoms and functioning, and trajectories over time. Methods: Data from the Trajectories of Outcome in Neurological Conditions study were analyzed by Rasch analysis, structural equation and group-based trajectory models. Results: Fatigue was reported by 97.8% on Neurological Fatigue Index-MND (NFI-MND) and 96.4% on Numeric Rating Scale Fatigue among 1058 pwALS: mean age 65 (range 20-90); mean duration 23 months (range 0-301); 60.7% male; onset 26.5% Bulbar, 71.5% Limb and 2.0% Respiratory. Mean (metric) level on NFI-MND was 12.8 (SD 5.3; range 0-24). Cut-points on the NFI-MND of 10 and 15 divided fatigue into mild (27.3%); moderate (36.1%) and severe (36.2%). Structural equation modeling showed that function, cognition, spasticity, dyspnea and pain have descending order of effect. Over average 11.6 months follow-up, 60.5% had stable fatigue, 23.8% increased fatigue level, while 15.8% showed declining fatigue. Trajectory analysis showed three groups, low, average and high fatigue. Those with low trajectories had less spasticity, worry, cognitive problems, as well as better functioning, longer duration and were less likely to be male. High fatigue trajectory was associated with worse spasticity, cognition and anxiety. Conclusions: Fatigue is extremely common among pwALS, thus more work is required on fatigue management. In addition to treating fatigue itself, the current study shows that targeting cognition, spasticity, dyspnea and pain might be fruitful.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-12"},"PeriodicalIF":0.0,"publicationDate":"2025-07-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144700465","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}

引用次数: 0

Patient reported outcome measures require scale metrification and quantified precision: evidence from the assessment of breathlessness in people with ALS/MND. 患者报告的结果测量需要尺度度量化和量化精度：来自ALS/MND患者呼吸困难评估的证据。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-07-20 DOI: 10.1080/21678421.2025.2533870

Carolyn A Young, Amina Chaouch, Christopher J Mcdermott, Ammar Al-Chalabi, Suresh Kumar Chhetri, Nicola Waters, Richard Buccleuch, Roger J Mills, Alan Tennant

{"title":"Patient reported outcome measures require scale metrification and quantified precision: evidence from the assessment of breathlessness in people with ALS/MND.","authors":"Carolyn A Young, Amina Chaouch, Christopher J Mcdermott, Ammar Al-Chalabi, Suresh Kumar Chhetri, Nicola Waters, Richard Buccleuch, Roger J Mills, Alan Tennant","doi":"10.1080/21678421.2025.2533870","DOIUrl":"https://doi.org/10.1080/21678421.2025.2533870","url":null,"abstract":"Introduction: Precision (how closely repeated measures match) and responsiveness (ability to detect change over time) are critical properties of patient reported outcome measures (PROMs). Smallest Detectable Difference (SDD) is a useful statistic regarding precision; Minimal Detectable Change (MDC) and Minimal Important Change (MIC) assess responsiveness. Methods: We examined measurement properties of Numeric Rating Scale for Breathlessness, ALSFRS-R respiratory subscale and Dyspnea-12, contributed by participants in the Trajectories of Outcome in Neurological Conditions-ALS study. Rasch analysis converts ordinal scale data to interval equivalents. Results: Data from 1120 people with ALS showed ALSFRS-R Respiratory is only valid as ordinal data. The NRS Breathlessness requires computation from a wider NRS set for Rasch analysis; its SDD is 3.2, MDC 2.59, MIC 2.39, with score range of 0-10. The Dyspnea-12 has SDD 7.0, MDC 6.14, MIC 4.5, with score range of 0-36. The %MDC, indicating smallest change detectable above measurement error as % of scale range, is superior for the Dyspnea-12 (17.1%) compared to the NRS Breathlessness (25.9%). Another advantage of Dyspnea-12 is transformation of raw ordinal to interval equivalent data using published conversion tables. Both NRS and Dyspnea-12 have disadvantages of MIC < MDC. Conclusions: Accurate measurement underpins optimal clinical decision making and high-quality research. Informed choice of PROMs reduces risk of misinterpreting clinical and research data. Patients want PROMs which they feel give an accurate account of their progression when participating in research and communicating with their clinical team. The Dyspnea-12 is preferrable for clinical and research use based on its psychometric properties.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-7"},"PeriodicalIF":0.0,"publicationDate":"2025-07-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144676657","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}

引用次数: 0

Utilization of patient-reported outcome measures in amyotrophic lateral sclerosis management: a cross-sectional study of Spanish neurologists. 肌萎缩性侧索硬化症管理中患者报告结果测量的使用：西班牙神经科医生的横断面研究。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-07-11 DOI: 10.1080/21678421.2025.2523940

Beatriz Vélez-Gómez, Macarena Cabrera-Serrano, Carmen Paradas

{"title":"Utilization of patient-reported outcome measures in amyotrophic lateral sclerosis management: a cross-sectional study of Spanish neurologists.","authors":"Beatriz Vélez-Gómez, Macarena Cabrera-Serrano, Carmen Paradas","doi":"10.1080/21678421.2025.2523940","DOIUrl":"https://doi.org/10.1080/21678421.2025.2523940","url":null,"abstract":"Objective: Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that significantly impacts quality of life. Patient-Reported Outcome Measures (PROMs) offer a patient-centered approach by capturing self-reported assessments of symptoms and well-being. Despite their recognized value, PROM integration into ALS management remains inconsistent. This study evaluates the attitudes, practices, and barriers experienced by Spanish neurologists regarding PROM use in ALS care. Methods: A cross-sectional survey was distributed to Spanish neurologists specializing in neuromuscular disorders. The questionnaire assessed familiarity with and use of PROMs, as well as perceived benefits and barriers to their implementation. Statistical analysis included descriptive statistics, group comparisons, and exploratory factor analysis (EFA) to identify underlying factors influencing PROM use. Results: Among 60 neurologists surveyed, 93.3% were familiar with PROMs, yet only 18.3% used them routinely. PROM use did not vary significantly with years of experience, type of clinical setting, exclusive dedication to neuromuscular disorders, or the percentage of time spent on patient care. The only variable approaching significance was the number of ALS patients managed daily, with higher patient volumes associated with more frequent PROM use. Over 70% of non-users cited limited consultation time as a barrier; however, factor analysis indicated that time constraints were not a substantial limitation. PROMs were valued for supporting clinical decision-making, monitoring disease progression, and improving patient engagement. Conclusions: While PROMs are widely recognized for their potential in ALS care, barriers hinder their use. Targeted training, simplified tools, and culturally adapted PROMs are needed to facilitate broader adoption and improve outcomes.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-9"},"PeriodicalIF":0.0,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144610445","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}

引用次数: 0