样式: 排序: IF: - GO 导出 标记为已读
-
Correction Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-04-17
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration (Vol. 25, No. 3-4, 2024)
-
Cognitive reserve in ALS: the role of occupational skills and requirements Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-04-09 Emma Rhodes, Sebleh Alfa, Hannah A. Jin, Lauren Massimo, Lauren Elman, Defne Amado, Michael Baer, Colin Quinn, Corey T. McMillan
Amyotrophic Lateral Sclerosis (ALS) is a heterogeneous neurodegenerative condition featuring variable degrees of motor and cognitive impairment. We assessed the impact of specific, empirically deri...
-
Case-control study of environmental toxins and risk of amyotrophic lateral sclerosis involving the national ALS registry Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-04-09 Evelyn O. Talbott, Angela M. Malek, Vincent C. Arena, Fan Wu, Kristen Steffes, Ravi K. Sharma, Jeanine Buchanich, Judith R. Rager, Todd Bear, Caroline A. Hoffman, David Lacomis, Chris Donnelly, Jocelyn Mauna, John E. Vena
Objective: Neurotoxic chemicals are suggested in the etiology of amyotrophic lateral sclerosis (ALS). We examined the association of environmental and occupational risk factors including persistent...
-
Assessing pulmonary function in ALS using electrical impedance tomography Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-04-04 Seward B. Rutkove, Courtney E. McIlduff, Elijah Stommel, Sean Levy, Christy Smith, Hilda Gutierrez, Sarah Verga, Soleil Samaan, Chebet Yator, Ajitesh Nanda, Lisa Pastel, Allaire Doussan, Kathy Phipps, Ethan Murphy, Ryan Halter
Objective: We sought to determine whether thoracic electrical impedance tomography (EIT) could characterize pulmonary function in amyotrophic lateral sclerosis (ALS) patients, including those with ...
-
Incidence of amyotrophic lateral sclerosis before and during the COVID-19 pandemic: evidence from an 8-year population-based study in Central Italy based on healthcare utilization databases Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-04-01 Federico Maria Sopranzi, Andrea Faragalli, Marco Pompili, Flavia Carle, Rosaria Gesuita, Maria Gabriella Ceravolo
Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disorder with a high multidimensional burden, with an obscure etiopathogenesis.We designed a longitudinal, population-based study of...
-
Artificial intelligence for screening and diagnosis of amyotrophic lateral sclerosis: a systematic review and meta-analysis Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-04-02 Tungki Pratama Umar, Nityanand Jain, Manthia Papageorgakopoulou, Rahma Sameh Shaheen, Jehad Feras Alsamhori, Muhammad Muzzamil, Andrejs Kostiks
Amyotrophic lateral sclerosis (ALS) is a rare and fatal neurological disease that leads to progressive motor function degeneration. Diagnosing ALS is challenging due to the absence of a specific de...
-
Residential exposure associations with ALS risk, survival, and phenotype: a Michigan-based case-control study Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-04-01 Stephen A. Goutman, Jonathan Boss, Dae Gyu Jang, Caroline Piecuch, Hasan Farid, Madeleine Batra, Bhramar Mukherjee, Eva L. Feldman, Stuart A. Batterman
Background: Environmental exposures impact amyotrophic lateral sclerosis (ALS) risk and progression, a fatal and progressive neurodegenerative disease. Better characterization of these exposures is...
-
Patient engagement in research: lessons learned from CAPTURE ALS, a longitudinal observational ALS study Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-03-22 Shelagh K. Genuis, Westerly Luth, Claire Magnussen, Christine Vande Velde, David Taylor, CAPTURE ALS Participant Partner Advisory Council, Wendy S. Johnston
Objective: There are compelling ethical and practical reasons for patient engagement in research (PEIR), however, evidence for best practices remains limited. We investigated PEIR as implemented in...
-
Incidence of amyotrophic lateral sclerosis in Chile Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-03-20 Patricia Lillo, Pedro Zitko, Gladys Godoy-Reyes, Gabriela Asenjo, David Sáez, Gonzalo Cea, Pamela Navarrete, Daniel Valenzuela, Ricardo Hughes, Mark Heverin, Giancarlo Logroscino, Orla Hardiman
This study aimed to estimate amyotrophic lateral sclerosis (ALS) incidence and survival rates in the Metropolitan region of Chile.We conducted a cohort study of ALS cases in the Metropolitan Region...
-
Psychometric properties of the Russian version of the Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-03-20 Mansur A. Kutlubaev, Daria K. Areprintseva, Ratko Radakovic, Ekaterina V. Pervushina
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative condition with observable cognitive and behavioral impairment. The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) is a tool develope...
-
Longitudinal comparison of the self-administered ALSFRS-RSE and ALSFRS-R as functional outcome measures in ALS Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-03-19 M. Kelley Erb, Narghes Calcagno, Roland Brown, Katherine M. Burke, Zoe A. Scheier, Amrita S. Iyer, Alison Clark, Max P. Higgins, Mackenzie Keegan, Anoopum S. Gupta, Stephen A. Johnson, Sheena Chew, James D. Berry
Objective: Test the feasibility, adherence rates and optimal frequency of digital, remote assessments using the ALSFRS-RSE via a customized smartphone-based app. Methods: This fully remote, longitu...
-
Dyspnea (breathlessness) in amyotrophic lateral sclerosis/motor neuron disease: prevalence, progression, severity, and correlates Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-03-11 Carolyn A. Young, Amina Chaouch, Christopher J. Mcdermott, Ammar Al-Chalabi, Suresh K. Chhetri, Kevin Talbot, Timothy Harrower, Richard W. Orrell, Joe Annadale, Oliver C. Hanemann, Antonio Scalfari, Alan Tennant, Roger Mills, ON BEHALF OF THE TONIC-ALS STUDY GROUP
Objective: Dyspnea, or breathlessness, is an important symptom in amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). We examined the measurement properties of the Dyspnea-12. Methods: Ra...
-
Genetic and in silico analysis of Indian sporadic young onset patient with amyotrophic lateral sclerosis Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-03-07 Saileyee Roychowdhury, Deepika Joshi, Vinay Kumar Singh, Mohammed Faruq, Parimal Das
Background: Amyotrophic lateral sclerosis (ALS) is an old onset devastating neurodegenerative disorder. Young-onset ALS cases especially sporadic ones who are between 25 and 45 years are rarely aff...
-
Correction Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-03-06
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration (Ahead of Print, 2024)
-
What is the extent of reliability and validity evidence for screening tools for cognitive and behavioral change in people with ALS? A systematic review Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-28 Lyndsay Didcote, Silia Vitoratou, Ammar Al-Chalabi, Laura H. Goldstein
Objective: This systematic review provides an updated summary of the existing literature on the validity of screening tools for cognitive and behavioral impairment in people with Amyotrophic Latera...
-
Improving the measurement properties of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R): deriving a valid measurement total for the calculation of change Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-03-01 Carolyn A Young, Amina Chaouch, Christopher J Mcdermott, Ammar Al-Chalabi, Suresh K Chhetri, Kevin Talbot, Andrea Malaspina, Roger Mills, Alan Tennant
The Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) total score is a widely used measure of functional status in Amyotrophic Lateral Sclerosis/Motor Neuron Disease (ALS), b...
-
Medication use and risk of amyotrophic lateral sclerosis: using machine learning for an exposome-wide screen of a large clinical database Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-03-01 Ran S Rotem, Andrea Bellavia, Sabrina Paganoni, Marc G Weisskopf
Accumulating evidence suggests that non-genetic factors have important etiologic roles in amyotrophic lateral sclerosis (ALS), yet identification of specific culprit factors has been challenging. M...
-
The ALSFRS-R Summit: a global call to action on the use of the ALSFRS-R in ALS clinical trials Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-23 Angela Genge, Jesse M. Cedarbaum, Jeremy Shefner, Adriano Chio, Ammar Al-Chalabi, Philip Van Damme, Chris McDermott, Jonathan Glass, James Berry, Ruben P.A. van Eijk, Christina Fournier, Julian Grosskreutz, Jinsy Andrews, Vanessa Bertone, Tommy M Bunte, Mathias Couillard, Cathy Cummings, Gale Kittle, John Polzer, Kristiana Salmon, Corey Straub, Leonard H. van den Berg
The Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) was developed more than 25 years ago as an instrument to monitor functional change over time in patients with ALS. It has since be...
-
Geographical distribution of clinical trials in amyotrophic lateral sclerosis: a scoping review Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-23 Beliu García-Parra, Josep M. Guiu, Mónica Povedano, Eduardo L. Mariño, Pilar Modamio
Introduction: Clinical trials location is determined by many factors, including the availability of patient populations, regulatory environment, scientific expertise, and cost considerations. In cl...
-
Correction Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-21
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration (Vol. 25, No. 3-4, 2024)
-
Rurality modifies the association between symptoms and the diagnosis of amyotrophic lateral sclerosis Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-14 Alexander A. Hart, Andrea Swenson, Nandakumar S. Narayanan, Jacob E. Simmering
We utilized national claims-based data to identify the change in odds of diagnosis of ALS following possible-ALS-symptoms-and whether the change varies in urban/rural areas.Insurance claims were ob...
-
Prognosis of amyotrophic lateral sclerosis patients after tracheostomy invasive ventilation in Korea Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-10 Jong-Su Kim, Minae Park, Sojeong Park, Juhee Chae, Yoon-Ho Hong, Kyung Seok Park, Jung-Joon Sung, Seok-Jin Choi
Background: Tracheostomy invasive ventilation (TIV) is applied to a subset of amyotrophic lateral sclerosis (ALS) patients; however, its frequency and impact on prognosis vary across countries. Met...
-
Supporting people with Motor Neuron Disease (MND) to make decisions about gastrostomy feeding tube placement: a survey of UK healthcare professionals’ practice and beliefs Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-09 Sean White, Alicia O’Cathain, Vanessa Halliday, Michael Bradburn, Christopher J. McDermott
Understand the practice and beliefs of healthcare professionals (HCPs) supporting the decision-making of people with MND (pwMND) about gastrostomy placement, including identifying differences betwe...
-
ALSUntangled #74: Withania Somnifera (Ashwagandha) Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-06 Sartaj Jhooty, Paul Barkhaus, Andrew Brown, Javier Mascias Cadavid, Gregory T. Carter, Jesse Crayle, Terry Heiman-Patterson, Xiaoyan Li, Elise Mallon, Christopher Mcdermott, Tasnim Mushannen, Gary Pattee, Dylan Ratner, Paul Wicks, Martina Wiedau, Richard Bedlack
ALSUntangled reviews alternative and off-label treatments on behalf of people with ALS (PALS) who ask about them. Here, we review withania somnifera (WS) commonly known as ashwagandha or winter che...
-
ALSFRS-R decline rate prior to baseline is not useful for stratifying subsequent progression of functional decline Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-07 Tatsuto Hamatani, Naoki Atsuta, Fumiya Sano, Ryoichi Nakamura, Yukikazu Hayashi, Gen Sobue
One of the difficulties in developing a novel drug for patients with amyotrophic lateral sclerosis (ALS) is the significant variation in the clinical course. To control this variation, a 12-week ru...
-
Verbal expressive language minimally affected in non-demented people living with amyotrophic lateral sclerosis Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-02-02 Elisabeth Kasper, Anna G. M. Temp, Verena Köckritz, Lisa Meier, Judith Machts, Stefan Vielhaber, Andreas Hermann, Johannes Prudlo
Objective: Language dysfunction is one of the most common cognitive impairments in amyotrophic lateral sclerosis (ALS). Although discourse capacities are essential for daily functioning, verbal exp...
-
Correction Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration (Vol. 25, No. 1-2, 2024)
-
Peripheral immunity relate to disease progression and prognosis in amyotrophic lateral sclerosis Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-25 Qirui Jiang, Qianqian Wei, Lingyu Zhang, Tianmi Yang, Junyu Lin, Yi Xiao, Chunyu Li, Yanbing Hou, Ruwei Ou, Kuncheng Liu, Bi Zhao, Ying Wu, Xiaohui Lai, Huifang Shang
Background: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Abnormalities in the peripheral immune system in ALS have been paid attention; however, the results of changes ...
-
A comparison between bioelectrical impedance analysis and air-displacement plethysmography in assessing fat-free mass in patients with motor neurone diseases: a cross-sectional study Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-24 Mark R. Janse van Mantgem, Maaike L. Soors D’Ancona, Myrte Meyjes, Leonard H. Van Den Berg, Elles Steenhagen, Annemieke Kok, Ruben P. A. Van Eijk
To determine the validity of bioelectrical impedance analysis (BIA) in quantifying fat-free mass (FFM) compared to air-displacement plethysmography (ADP) in patients with a motor neurone disease (M...
-
Plasma neurofilament light levels show elevation two years prior to diagnosis of amyotrophic lateral sclerosis in the UK Biobank Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Erin N. Smith, Jonghun Lee, Daria Prilutsky, Stephen Zicha, Zemin Wang, Steve Han, Neta Zach
Amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative disease with profound unmet need. In patients carrying genetic mutations, elevations in neurofilament light (NfL) have been s...
-
Prognostic value of motor and extramotor involvement in ALS Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Vincent Kabir, Fouke Ombelet, Frederik Hobin, Nikita Lamaire, Joke De Vocht, Philip Van Damme
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder resulting in upper and lower motor neuron loss. ALS often has a focal onset of weakness, which subsequently spreads to other body...
-
Development, reliability, validity, and acceptability of the remote administration of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Debbie Gray, Rosemary Lesley, Emily J. Mayberry, Luke Williams, Caroline McHutchison, Judith Newton, Suvankar Pal, Siddharthan Chandran, Sarah E. MacPherson, Sharon Abrahams, MND Consortium
ALS clinical care and research has changed dramatically since the COVID-19 pandemic, accelerating the need for cognitive assessments to be adapted for remote use.To develop the remote administratio...
-
FUS gene mutation in amyotrophic lateral sclerosis: a new case report and systematic review Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Xin Xiao, Min Li, Zhi Ye, Xiaoyan He, Jun Wei, Yunhong Zha
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease associated with upper and lower motor neuron degeneration and necrosis, characterized by progressive muscle weakness, atroph...
-
Racial disparities in ALS diagnostic delay: a single center’s experience and review of potential contributing factors Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Shanshan Chen, Demetrius Carter, Paula Burke Brockenbrough, Stephen Cox, Kelly Gwathmey
Outcomes for amyotrophic lateral sclerosis (ALS) patients are improved with prompt diagnosis, earlier initiation of disease-modifying treatments, and participation in a multidisciplinary clinic. We...
-
Genetic overlap between ALS and other neurodegenerative or neuromuscular disorders Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Cathrine Goberg Olsen, Øyvind Løvold Busk, Øystein Lunde Holla, Kristian Tveten, Trygve Holmøy, Ole-Bjørn Tysnes, Helle Høyer
In Norway, 89% of patients with Amyotrophic lateral sclerosis (ALS) lacks a genetic diagnose. ALS genes and genes that cause other neuromuscular or neurodegenerative disorders extensively overlap. ...
-
Analysis of normal C9orf72 repeat length as possible disease modifier in amyotrophic lateral sclerosis Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Silvia Peverelli, Alberto Brusati, Valeria Casiraghi, Marta Nice Sorce, Sabrina Invernizzi, Serena Santangelo, Claudia Morelli, Federico Verde, Vincenzo Silani, Nicola Ticozzi, Antonia Ratti
The C9orf72 hexanucleotide repeat (HR) expansion is the main genetic cause of amyotrophic lateral sclerosis (ALS), with expansion size from 30 to >4000 units. Normal C9orf72 HR length is polymorphi...
-
Optineurin in patients with Amyotrophic Lateral Sclerosis associated to atypical Parkinsonism in Tunisian population Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 I. Kacem, I. Sghaier, S. Peverelli, Y. Abida, H. Ben Brahim, A. Ratti, A. Nasri, N. Ticozzi, V. Silani, R. Gouider
Amyotrophic Lateral Sclerosis (ALS) is a heterogeneous disorder and the phenotypic variability goes far beyond the used clinical stratification parameter. Evidence has emerged that ALS may coexist ...
-
Non-motor symptoms in amyotrophic lateral sclerosis Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Ali Shojaie, Ahmad Al Khleifat, Sarah Opie-Martin, Payam Sarraf, Ammar Al-Chalabi
While motor symptoms are well-known in ALS, non-motor symptoms are often under-reported and may have a significant impact on quality of life. In this study, we aimed to examine the nature and exten...
-
LAENALS: epidemiological and clinical features of amyotrophic lateral sclerosis in Latin America Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Beatriz Vélez-GóMEZ, Abayuba Perna, Cristina Vazquez, Carlos Ketzoian, Patricia Lillo, Gladys Godoy-Reyes, David Sáez, Tatiana Zaldivar Vaillant, Joel Víctor Gutiérrez Gil, Gloria Esther Lara-Fernández, Mónica Povedano, Mark Heverin, Robert McFarlane, Giancarlo Logroscino, Orla Hardiman
The Latin American Epidemiologic study of ALS (LAENALS) aims to gather data on ALS epidemiology, phenotype, and risk factors in Cuba, Chile, and Uruguay, to understand the impact of genetic and env...
-
Assessment of risk of ALS conferred by the GGGGCC hexanucleotide repeat expansion in C9orf72 among first-degree relatives of patients with ALS carrying the repeat expansion Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Iris F. Van Wijk, Ruben P.A. Van Eijk, Loes Van Boxmeer, Henk-Jan Westeneng, Michael A. Van Es, Wouter Van Rheenen, Leonard H. Van Den Berg, Marinus J.C. Eijkemans, Jan H. Veldink
We aimed to estimate the age-related risk of ALS in first-degree relatives of patients with ALS carrying the C9orf72 repeat expansion.We included all patients with ALS carrying a C9orf72 repeat exp...
-
Misdiagnosis of amyotrophic lateral sclerosis in clinical practice in Europe and the USA: a patient chart review and physician survey Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Namita A. Goyal, Kerina Bonar, Natasa Savic, Raphaëlle Beau Lejdstrom, Jack Wright, Jennifer Mellor, Christopher McDermott
Delays in amyotrophic lateral sclerosis (ALS) diagnosis can result in compromised disease management and unnecessary costs. We examined the extent of ALS misdiagnosis in the US and Europe.Data were...
-
A brief report on juvenile amyotrophic lateral sclerosis cases in the United States National ALS Registry: 2010–2018 Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Jaime Raymond, Jasmine Berry, Edward J. Kasarskis, Theodore Larson, D. Kevin Horton, Paul Mehta
Juvenile ALS (jALS) is a rare form of ALS, defined as symptom onset before age 25. This report describes the demographic characteristics of confirmed and likely jALS cases in a large cohort of ALS ...
-
Describing and characterising variability in ALS disease progression Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Muzammil Arif Din Abdul Jabbar, Ling Guo, Yang Guo, Zachary Simmons, Erik P. Pioro, Savitha Ramasamy, Crystal Jing Jing Yeo
Decrease in the revised ALS Functional Rating Scale (ALSFRS-R) score is currently the most widely used measure of disease progression. However, it does not sufficiently encompass the heterogeneity ...
-
Neurodegenerative biomarkers outperform neuroinflammatory biomarkers in amyotrophic lateral sclerosis Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Ulf Kläppe, Stefan Sennfält, Anikó Lovik, Anja Finn, Ulrika Bofaisal, Henrik Zetterberg, Kaj Blennow, Fredrik Piehl, Ivan Kmezic, Rayomand Press, Kristin Samuelsson, Anna Månberg, Fang Fang, Caroline Ingre
To describe the diagnostic and prognostic performance, and longitudinal trajectories, of potential biomarkers of neuroaxonal degeneration and neuroinflammation in amyotrophic lateral sclerosis (ALS...
-
Integrated analysis of transcriptomic and proteomic alterations in mouse models of ALS/FTD identify early metabolic adaptions with similarities to mitochondrial dysfunction disorders Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Anna Matveeva, Orla Watters, Ani Rukhadze, Niraj Khemka, Debora Gentile, Ivan Fernandez Perez, Irene Llorente-Folch, Cliona Farrell, Elide Lo Cacciato, Joshua Jackson, Antonia Piazzesi, Lena Wischhof, Ina Woods, Luise Halang, Marion Hogg, Amaya Garcia Muñoz, Eugène T. Dillon, David Matallanas, Ingrid Arijs, Diether Lambrechts, Daniele Bano, Niamh M. C. Connolly, Jochen H. M. Prehn
Sporadic and familial amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disease that results in loss of motor neurons and, in some patients, associates with frontotempora...
-
Clinical epidemiology of amyotrophic lateral sclerosis in Liguria, Italy: a ten year follow up study Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Corrado Cabona, Pilar Maria Ferraro, Carlo Scialò, Monica Bandettini Di Poggio, Giovanni Novi, Chiara Gemelli, Manuela Vignolo, Fabrizio Rao, Marina Capovilla, Maura Marogna, Paola Mandich, Paola Origone, Angelo Schenone, Claudia Caponnetto
This article presents an updated analysis of the LIGALS register, a prospective study conducted over a ten-year period (2009-2018) in Liguria, Italy, aimed at evaluating the incidence, prevalence, ...
-
The importance of understanding minimal important difference for ALSFRS-R: A reply to ‘Do we really need to calculate a minimal important difference for ALSFRS-R?’ https://doi.org/10.1080/21678421.2023.2248199 Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Christina N. Fournier, Jonathan D. Glass
Published in Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration (Vol. 25, No. 1-2, 2024)
-
Harmonized standard operating procedures for administering the ALS functional rating scale-revised Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-23 Jeremy M. Shefner, Tommy Bunte, Gale Kittle, Angela Genge, Leonard H van den Berg
The ALS Functional Rating Scale-Revised is the most commonly used primary outcome measure in current ALS clinical trials. While rigorous training and certification is generally recognized as critic...
-
Specialized multidisciplinary care improves ALS survival in Belgium: a population-based retrospective study Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-19 Frederik Hobin, Joke De Vocht, Nikita Lamaire, Hilde Beyens, Fouke Ombelet, Philip Van Damme
ALS is a neurodegenerative disease characterized by loss of motor neurons, resulting in progressive weakness and wasting of muscles. The average survival time is 2–5 years, mostly due to respirator...
-
Diagnostic utility of Gold Coast criteria for amyotrophic lateral sclerosis in Asia Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-16 Ryo Otani, Kazumoto Shibuya, Toshio Shimizu, Takamasa Kitaoji, Yu-Ichi Noto, Kota Bokuda, Hideki Kimura, Tomoki Suichi, Keigo Nakamura, Hiroki Kano, Marie Morooka, Yuya Aotsuka, Moeko Ogushi, Sonoko Misawa, Satoshi Kuwabara
Objective: This study aimed to reveal the diagnostic utility of Gold Coast (GC) criteria in Japanese patients with amyotrophic lateral sclerosis (ALS) by comparing the sensitivity/specificity with ...
-
A report of resources used by clinicians in the UK to support motor neuron disease genomic testing Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2024-01-02 Jade Howard, Hilary L. Bekker, Christopher J. Mcdermott, Alisdair Mcneill
Genetic testing is a key decision-making point for people with motor neuron disease (MND); to establish eligibility for clinical trials, better understand the cause of their condition, and confirm ...
-
Time from amyotrophic lateral sclerosis symptom onset to key disease milestones: analysis of data from a multinational cross-sectional survey Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2023-12-29 Paulos Gebrehiwet, Johan Brekke, Stacy A. Rudnicki, Jennifer Mellor, Jack Wright, Lucy Earl, Nathan Ball, Halima Iqbal, Owen Thomas, Giorgio Castellano
To determine the average time from Amyotrophic Lateral Sclerosis (ALS) symptom onset to 11 pre-defined milestones, overall and according to ALS progression rate and geographic location.Data were dr...
-
ALSUntangled #73: Lion’s Mane Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2023-12-23 Maya Muhanna, Issac Lund, Mark Bromberg, Paul Wicks, Michael Benatar, Benjamin Barnes, Kaitlyn Pierce, Dylan Ratner, Andrew Brown, Tulio Bertorini, Paul Barkhaus, Greg Carter, Javier Mascias Cadavid, Christopher McDermott, Jonathan D Glass, Gary Pattee, Carmel Armon, Richard Bedlack, Xiaoyan Li
Lion’s Mane (Hericium erinaceus) has historically been used as traditional medicine in Asia and Europe for its potential benefits in fighting infection and cancer. It has gained interest in the neu...
-
Premorbid lipid levels and long-term risk of ALS—a population-based cohort study Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2023-12-20 Anders Myhre Vaage, Jūratė Šaltytė Benth, Haakon E. Meyer, Trygve Holmøy, Ola Nakken
To assess the temporal relationship between premorbid lipid levels and long-term amyotrophic lateral sclerosis (ALS) risk.From Norwegian cardiovascular health surveys (1974–2003), we collected info...
-
What, how and when do families communicate about ALS? A qualitative exploration of parents’ and children’s perceptions Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2023-12-09 Marion Sommers-Spijkerman, Anna Stukker, Melinda S. Kavanaugh, Marjolijn Ketelaar, Johanna M. A. Visser-Meily, Anita Beelen
Objectives: In families with a parent diagnosed with amyotrophic lateral sclerosis (ALS), children’s adaptation depends among others on how their parents communicate with them about the disease and...
-
ALS Health care provider wellness Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2023-12-09 Gregory Hansen, Sarah Burton-MacLeod, Kerri Lynn Schellenberg
Interest in health care provider (HCP) wellness and burnout is increasing; however, minimal literature explores HCP wellness in the context of Amyotrophic Lateral Sclerosis (ALS) care.We sought to ...
-
Predicting amyotrophic lateral sclerosis (ALS) progression with machine learning Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2023-12-05 Muzammil Arif Din Abdul Jabbar, Ling Guo, Sonakshi Nag, Yang Guo, Zachary Simmons, Erik P. Pioro, Savitha Ramasamy, Crystal Jing Jing Yeo
To predict ALS progression with varying observation and prediction window lengths, using machine learning (ML).We used demographic, clinical, and laboratory parameters from 5030 patients in the Poo...
-
Digital markers of motor speech impairments in spontaneous speech of patients with ALS-FTD spectrum disorders Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2023-12-05 Sanjana Shellikeri, Sunghye Cho, Sharon Ash, Carmen Gonzalez-Recober, Corey T. Mcmillan, Lauren Elman, Colin Quinn, Defne A. Amado, Michael Baer, David J. Irwin, Lauren Massimo, Christopher A. Olm, Mark Y. Liberman, Murray Grossman, Naomi Nevler
To evaluate automated digital speech measures, derived from spontaneous speech (picture descriptions), in assessing bulbar motor impairments in patients with ALS-FTD spectrum disorders (ALS-FTSD).A...
-
ALSUntangled #72: Insulin Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2023-11-28 Andrew Brown, Carmel Armon, Paul Barkhaus, Morgan Beauchamp, Tulio Bertorini, Mark Bromberg, Javier Mascias Cadavid, Gregory T. Carter, Jesse Crayle, Eva L. Feldman, Terry Heiman-Patterson, Sartaj Jhooty, Alexandra Linares, Xiaoyan Li, Elise Mallon, Christopher Mcdermott, Tasnim Mushannen, George Nathaniel, Gary Pattee, Kaitlyn Pierce, Dylan Ratner, Lenka Slactova, Paul Wicks, Richard Bedlack
ALSUntangled reviews alternative and off-label treatments for people living with amyotrophic lateral sclerosis (PALS). Here we review insulin, which has at least one plausible mechanism for slowing...
-
Amyotrophic lateral sclerosis: exploring pathophysiology in the context of treatment Amyotroph. Lateral Scler. Frontotemporal Degener. (IF 2.8) Pub Date : 2023-11-24 Angela Genge, Steven Wainwright, Christine Vande Velde
Amyotrophic lateral sclerosis (ALS) is a complex, neurodegenerative disorder in which alterations in structural, physiological, and metabolic parameters act synergistically. Over the last decade th...