Amyotrophic lateral sclerosis & frontotemporal degeneration最新文献_第3页

Motor neuron diseases are not exclusively motor; the SSR paradigm.

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-01-29 DOI: 10.1080/21678421.2025.2458694

Marianna Papadopoulou, Maria-Ioanna Stefanou, Stella Fanouraki, Christos Moschovos, Eleni Bakola, Stavroula Salakou, Vasiliki Zouvelou, George K Papadimas, Georgios Tsivgoulis

{"title":"Motor neuron diseases are not exclusively motor; the SSR paradigm.","authors":"Marianna Papadopoulou, Maria-Ioanna Stefanou, Stella Fanouraki, Christos Moschovos, Eleni Bakola, Stavroula Salakou, Vasiliki Zouvelou, George K Papadimas, Georgios Tsivgoulis","doi":"10.1080/21678421.2025.2458694","DOIUrl":"https://doi.org/10.1080/21678421.2025.2458694","url":null,"abstract":"Motor Neuron Diseases (MNDs), familial and sporadic, are progressive neurodegenerative disorders that, for an extended period in the past, were considered purely motor disorders. During the course of the disease, however, some patients exhibit concomitant non-motor signs; thus, MNDs are currently perceived as multisystem disorders. Assessment of non-motor symptoms is usually performed clinically, although laboratory tests can also be routinely used to objectively evaluate these symptoms. Sympathetic Skin Response (SSR) is an example of a neurophysiological test that has been used in cases of Amyotrophic Lateral Sclerosis, Spinal Muscular Atrophy, and Monomelic Atrophy, mostly to assess Autonomic Nervous System (ANS) disorders. Dysautonomia affects quality of life and life expectancy, as it is involved in cardiovascular events and incidents of sudden death. SSR abnormalities are present even in subclinical involvement of the ANS in MNDs. In this review, we present published research examining SSR findings in various MNDs, and discuss the correlation of SSR findings with clinical symptoms and disease severity, as well as the potential sources of abnormal findings. The aim of this study is to raise clinician awareness of autonomic dysfunction in MNDs and present the benefits of SSR examination in patient care.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-10"},"PeriodicalIF":0.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143061604","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.

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub 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":"https://doi.org/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":"1-8"},"PeriodicalIF":0.0,"publicationDate":"2025-01-24","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

Maximizing the translational potential of neurophysiology in amyotrophic lateral sclerosis: a study on compound muscle action potentials. 肌萎缩性侧索硬化症神经生理学转化电位最大化：复合肌肉动作电位的研究。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-01-22 DOI: 10.1080/21678421.2024.2448540

Scott McKinnon, Zekai Qiang, Amy Keerie, Tyler Wells, Pamela J Shaw, James J P Alix, Richard J Mead

{"title":"Maximizing the translational potential of neurophysiology in amyotrophic lateral sclerosis: a study on compound muscle action potentials.","authors":"Scott McKinnon, Zekai Qiang, Amy Keerie, Tyler Wells, Pamela J Shaw, James J P Alix, Richard J Mead","doi":"10.1080/21678421.2024.2448540","DOIUrl":"https://doi.org/10.1080/21678421.2024.2448540","url":null,"abstract":"Mouse models of amyotrophic lateral sclerosis (ALS) enable testing of novel therapeutic interventions. However, treatments that have extended survival in mice have often failed to translate into human benefit in clinical trials. Compound muscle action potentials (CMAPs) are a simple neurophysiological test that measures the summation of muscle fiber depolarization in response to maximal stimulation of the innervating nerve. CMAPs can be measured in both mice and humans and decline with motor axon loss in ALS, making them a potential translational read-out of disease progression. We assessed the translational potential of CMAPs and ascertained time points when human and mouse data aligned most closely. We extracted data from 18 human studies and compared with results generated from SOD1G93A and control mice at different ages across different muscles. The relative CMAP amplitude difference between SOD1G93A and control mice in tibialis anterior (TA) and gastrocnemius muscles at 70 days of age was most similar to the relative difference between baseline ALS patient CMAP measurements and healthy controls in the abductor pollicis brevis (APB) muscle. We also found that the relative decline in SOD1G93A TA CMAP amplitude between 70 and 140 days was similar to that observed in 12 month human longitudinal studies in APB. Our findings suggest CMAP amplitudes can provide a \"translational window\", from which to make comparisons between the SOD1G93A model and human ALS patients. CMAPs are easy to perform and can help determine the most clinically relevant starting/end points for preclinical studies and provide a basis for predicting potential clinical effect sizes.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-9"},"PeriodicalIF":0.0,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143017300","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

Associations between water exposure and the development of amyotrophic lateral sclerosis: a matched case-control study. 水暴露与肌萎缩侧索硬化症发展之间的关系：一项匹配的病例对照研究。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-01-22 DOI: 10.1080/21678421.2025.2453450

Daniel Saucier, Mathieu Bélanger, Zikuan Liu, Eric Lavigne, Colleen O'Connell

{"title":"Associations between water exposure and the development of amyotrophic lateral sclerosis: a matched case-control study.","authors":"Daniel Saucier, Mathieu Bélanger, Zikuan Liu, Eric Lavigne, Colleen O'Connell","doi":"10.1080/21678421.2025.2453450","DOIUrl":"https://doi.org/10.1080/21678421.2025.2453450","url":null,"abstract":"Objective: Previous studies have hinted at an association between water exposure and the development of ALS. However, proximity measures to these water sources have been limited to questionnaires or large buffers due to a lack of fine geospatial measures. They also do not distinguish the various classes of hydrographic features. Thus, we created a robust database to investigate the association between proximity to water bodies at place of residence and the development of ALS.Methods: A matched (sex and year of birth) case-control study was conducted in New Brunswick, Canada from January 2003 to February 2021. Study population included 304 ALS patients and 1207 controls with their historical postal codes linked to spatial proximity datasets and air pollution index indicators (proxy measures for contamination by run-off).Results: Odds of ALS were not significantly associated with proximity to water bodies, even within a 250 m buffer from place of residence (Oceans: 1.10, 0.60-2.00 [95% CI], Reservoirs/Ponds/Lakes: 1.24, 0.47-3.30 [95% CI]). As for interaction models investigating proximity to potentially contaminated water bodies, none of the final fitted models observed an association between proximity to water bodies with indicators of potential run-off sources and the development of ALS.Conclusions: No significant association between proximity to water bodies at place of residence and the development of ALS were observed in the current study. Future studies should consider taking direct measurements of water quality or utilize geomaps of spraying activities and cyanobacteria blooms alongside proximity measures. Household water quality is another avenue to explore, particularly well water use.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-9"},"PeriodicalIF":0.0,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143017258","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

Stitching strength: things I've learned about hope and how I am trying to weave them into my in ALS practice. 缝合力量：我从希望中学到的东西，以及我如何将它们编织到我的ALS实践中。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-01-20 DOI: 10.1080/21678421.2025.2454903

Richard Bedlack

引用次数: 0

Rapid progression of amyotrophic lateral sclerosis after initiation of GLP-1 agonist: a case report. GLP-1激动剂启动后肌萎缩性侧索硬化症的快速进展：一例报告。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-01-07 DOI: 10.1080/21678421.2024.2446847

Kathleen Stolwyk, Ikjae Lee

引用次数: 0

Amyotrophic lateral sclerosis estimated prevalence cases from 2022 to 2030, data from the national ALS Registry. 肌萎缩性侧索硬化症的患病率估计从2022年到2030年，数据来自国家ALS登记处。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-01-03 DOI: 10.1080/21678421.2024.2447919

Paul Mehta, Jaime Raymond, Theresa Nair, Moon Han, Jasmine Berry, Reshma Punjani, Theodore Larson, Suraya Mohidul, D Kevin Horton

{"title":"Amyotrophic lateral sclerosis estimated prevalence cases from 2022 to 2030, data from the national ALS Registry.","authors":"Paul Mehta, Jaime Raymond, Theresa Nair, Moon Han, Jasmine Berry, Reshma Punjani, Theodore Larson, Suraya Mohidul, D Kevin Horton","doi":"10.1080/21678421.2024.2447919","DOIUrl":"https://doi.org/10.1080/21678421.2024.2447919","url":null,"abstract":"Objective: To estimate the projected number of ALS cases in the United States from 2022 to 2030. Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neuromuscular disease with no known cure. Because ALS is not a notifiable disease in the United States, the accurate ascertainment of prevalent ALS cases continues to be a challenge. To overcome this, the National ALS Registry (Registry) uses novel methods to estimate newly diagnosed and existing cases in the United States. Methods: We estimated ALS prevalence retrospectively from 2022 to 2024 and prospectively from 2025 to 2030 using prevalence obtained through previous CRC analyses on 2018 Registry data (the most current data available) to generate projected observed, missing, and total cases. Projected prevalent cases were then stratified by age, race, and sex. Results: The number of estimated ALS cases in 2022 was 32,893. By 2030, projected cases increase more than 10%, to 36,308. The largest increase occurs for the population ages 66 years and older, with a 25% increase (from 16,349 cases in 2022 to 20,438 cases in 2030). The projected number of cases classified as \"other race\" will increase by 15% (from 2,473 cases in 2022 to 2,854 cases in 2030). Conclusions: These estimates of projected ALS cases reflect anticipated changes in the underlying demographics of the United States. Our projections are likely an underestimation because emerging therapeutics and improved healthcare will improve survivability in this vulnerable population. These results should inform policy to more efficiently allocate resources for ALS patients and programs.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-6"},"PeriodicalIF":0.0,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142924151","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

Care trajectories and adherence to respiratory management recommendations in persons living with amyotrophic lateral sclerosis: a ten-year cohort study in a French tertiary university centre. 肌萎缩性侧索硬化症患者的护理轨迹和对呼吸管理建议的依从性：一项在法国高等教育大学中心进行的十年队列研究

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-01-03 DOI: 10.1080/21678421.2024.2447911

Pierre Tankéré, Estelle Cascarano, Christel Saint Raymond, Martial Mallaret, Cristina Toribio Ruiz, Eléonore Herquelot, Helene Denis, Mallory Cals Maurette, Renaud Tamisier, Jean Louis Pépin

{"title":"Care trajectories and adherence to respiratory management recommendations in persons living with amyotrophic lateral sclerosis: a ten-year cohort study in a French tertiary university centre.","authors":"Pierre Tankéré, Estelle Cascarano, Christel Saint Raymond, Martial Mallaret, Cristina Toribio Ruiz, Eléonore Herquelot, Helene Denis, Mallory Cals Maurette, Renaud Tamisier, Jean Louis Pépin","doi":"10.1080/21678421.2024.2447911","DOIUrl":"https://doi.org/10.1080/21678421.2024.2447911","url":null,"abstract":"Objective: This study determined real-life care trajectories before and after initiation of noninvasive ventilation (NIV) in patients with amyotrophic lateral sclerosis (ALS). Caregiver adherence to respiratory management recommendations and the associated survival rate of people with ALS were also assessed. Methods: Data were obtained from a tertiary center prospective ALS database that included 10 years of follow-up data for people with ALS. Results are presented numerically and with graphical time sequence analysis through K clustering (TAK) representation. Kaplan Meier and Cox models were used to determine survival and associated prognostic factors. Results: 109 patients with ALS patients were included; median [interquartile range] follow-up was 25.0 months [15.3-43.3]. During study timeframe patients had a median of 4.0 [2.0-6.0] clinical visits; death occurred in 54.1%. Median time between clinical visits was 3.9 [2.8-6.5] months, between arterial blood gases was 4.3 months [3.0-6.6], between spirometry testing was 5.8 months [4.1-8.2], and between nocturnal oximetry was 4.4 months [3.0-7.8]. Visualization of care trajectories TAK show marked heterogeneity in survival, time to NIV initiation, and time from NIV initiation to death. Mortality was correlated with NIV initiation and arterial carbon dioxide pressure increase. Conclusions: The current framework in ALS guidelines should be adapted to the ALS disease stage and individual patient characteristics. Understanding how subgroups of patients with ALS use healthcare services over time could help to highlight fragility areas and priorities in the allocation of care resources and implementation of best practices.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-9"},"PeriodicalIF":0.0,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142924155","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

Determinants and progression of stigma in amyotrophic lateral sclerosis/motor neuron disease. 肌萎缩性侧索硬化症/运动神经元疾病中柱头的决定因素和进展。

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2025-01-03 DOI: 10.1080/21678421.2024.2435969

Carolyn A Young, Amina Chaouch, Christopher J Mcdermott, Ammar Al-Chalabi, Suresh K Chhetri, Caroline Bidder, Elizabeth Edmonds, Cathy Ellis, Joe Annadale, Lisa Wilde, Basil Sharrack, Andrea Malaspina, Oliver Leach, Roger Mills, Alan Tennant

{"title":"Determinants and progression of stigma in amyotrophic lateral sclerosis/motor neuron disease.","authors":"Carolyn A Young, Amina Chaouch, Christopher J Mcdermott, Ammar Al-Chalabi, Suresh K Chhetri, Caroline Bidder, Elizabeth Edmonds, Cathy Ellis, Joe Annadale, Lisa Wilde, Basil Sharrack, Andrea Malaspina, Oliver Leach, Roger Mills, Alan Tennant","doi":"10.1080/21678421.2024.2435969","DOIUrl":"https://doi.org/10.1080/21678421.2024.2435969","url":null,"abstract":"Objective: Stigma in amyotrophic lateral sclerosis/motor neurone disease (ALS/MND) may be felt or enacted; felt stigma covers feeling devalued by the illness, whereas enacted stigma refers to being treated differently because of it. Stigma in ALS/MND has been shown to increase social withdrawal, worsen quality of life, and reduce use of assistive devices, so we explored prevalence and factors influencing stigma. Methods: Participants in the Trajectories of Outcome in Neurological Conditions-ALS study completed scales measuring stigma, fatigue, spasticity, functioning, mood, worry, self-esteem, and perceived health, as well as demographic information and symptoms like head drop or emotional lability. Following transformation to interval-scale estimates, data were analyzed by regression, structural equation modeling, and trajectory models. Results: Stigma was experienced by 83.5% of 1059 respondents. Worry, disease severity (King's stage ≥ 3), emotional lability, fatigue, spasticity, and bulbar onset increase stigma. In contrast, increasing age, living with spouse/partner, and greater self-esteem were associated with reduced stigma. Trajectory analysis over 30 months (N = 1049) showed three groups, the largest (70.2%) had high levels of stigma which significantly increased during follow-up. In a recently diagnosed subset of 347 participants, stigma was experienced early in the disease course (<7 months after diagnosis), and for 77.2% stigma significantly increased over time. Conclusions: Both felt and enacted stigma are frequently perceived by people living with ALS/MND. Younger people and those with bulbar onset, emotional lability, worry, fatigue, and spasticity, or at more advanced clinical stages, are at greater risk.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-11"},"PeriodicalIF":0.0,"publicationDate":"2025-01-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142924159","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

Characterization, epidemiology, and factors associated with evolution and survival in patients with amyotrophic lateral sclerosis in southeastern Spain, 2008-2021: a population-based study. 2008-2021年西班牙东南部肌萎缩性侧索硬化症患者的特征、流行病学和相关进化和生存因素：一项基于人群的研究

Amyotrophic lateral sclerosis & frontotemporal degeneration Pub Date : 2024-12-25 DOI: 10.1080/21678421.2024.2439454

Yaiza García-Ramírez, Juana-María Cayuela-Fuentes, María-Pilar Mira-Escolano, Luis-Alberto Maceda-Roldán, Eva Mikulasova, Cristina Oliva-López, Antonia Sánchez-Escámez, Pilar Ciller-Montoya, Joaquín A Palomar-Rodríguez

{"title":"Characterization, epidemiology, and factors associated with evolution and survival in patients with amyotrophic lateral sclerosis in southeastern Spain, 2008-2021: a population-based study.","authors":"Yaiza García-Ramírez, Juana-María Cayuela-Fuentes, María-Pilar Mira-Escolano, Luis-Alberto Maceda-Roldán, Eva Mikulasova, Cristina Oliva-López, Antonia Sánchez-Escámez, Pilar Ciller-Montoya, Joaquín A Palomar-Rodríguez","doi":"10.1080/21678421.2024.2439454","DOIUrl":"https://doi.org/10.1080/21678421.2024.2439454","url":null,"abstract":"Objective: To describe the epidemiology, characteristics, and factors associated with the evolution and survival in patients with amyotrophic lateral sclerosis (ALS) in a region of southeastern Spain.Methods: An observational study was carried out in people with a diagnosis of ALS in the period 2008-2021 who were registered in the Information System of Rare Diseases of the Region of Murcia (SIER). We calculated crude and standardized incidence rate (SIR) using European Standard Population of 2013 and point prevalence. The Kaplan-Meier method and the log-rank test were used to estimate and compare survival curves.Results: We identified 374 cases. The mean age at diagnosis was 66.5 ± 11.7 and 50.3% persons were spinal onset. Mean time from the onset of symptoms to diagnosis was 0.9 ± 1.0 years. The global SIR was 1.95/100,000 person-years (95%CI: 1.77-2.12), which was higher in men (ratio 1.34), and the point prevalence in 2021 was 4.57 per 100,000 (95% CI: 4.46-4.68). There were 297 deaths with a mean age of 69.8 ± 10.8. The median survival from clinical onset was 2 years (95%CI: 1.0-3.0). Factors associated with lower survival were bulbar onset (p < 0.001), older age at the onset of symptoms (p < 0.001), and the absence of riluzole treatment (p = 0.003).Conclusions: This study is one of few to evaluate the epidemiological, characteristics, and prognostic factors of ALS in Spain, with findings similar to previous population studies. The use of population-based registries offers reliable information on the magnitude, or evolution in these patients.","PeriodicalId":72184,"journal":{"name":"Amyotrophic lateral sclerosis & frontotemporal degeneration","volume":" ","pages":"1-13"},"PeriodicalIF":0.0,"publicationDate":"2024-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142900618","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