Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Volume 24, Issue 7-8 (2023)

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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Volume 24, Issue 7-8 (2023)

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Editorial

Editorial
Imaging extra-motor dysfunction in motor neuron disease: achievements and controversies
Peter Bede, Rangariroyashe H. Chipika & Ee Ling Tan
Pages: 559-561
Published online: 11 Jul 2023
First Page PreviewforImaging extra-motor dysfunction in motor neuron disease: achievements and controversies | Full Text | References | PDF (230.2 KB) | EPUB
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Review

Review Article
Non-motor symptoms in amyotrophic lateral sclerosis: lessons from Parkinson’s disease
Ali Shojaie, Silvia Rota, Ahmad Al Khleifat, K Ray Chaudhuri & Ammar Al-Chalabi
Pages: 562-571
Published online: 22 Jun 2023
AbstractforNon-motor symptoms in amyotrophic lateral sclerosis: lessons from Parkinson’s disease | Full Text | References | PDF (1 MB) | EPUB
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Open Access

Biomarkers

Review Article
MRI biomarkers for memory-related impairment in amyotrophic lateral sclerosis: a systematic review
Sadegh Ghaderi, Farzad Fatehi, Sanjay Kalra & Seyed Amir Hossein Batouli
Pages: 572-588
Published online: 19 Jul 2023
AbstractforMRI biomarkers for memory-related impairment in amyotrophic lateral sclerosis: a systematic review | Full Text | References | PDF (1.4 MB) | EPUB
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Article
PROSA—a multicenter prospective observational study to develop low-burden digital speech biomarkers in ALS and FTD
Johannes Tröger, Judith Baltes, Ebru Baykara, Elisabeth Kasper, Martha Kring, Nicklas Linz, Jessica Robin, Simona Schäfer, Anja Schneider & Andreas Hermann
Pages: 589-598
Published online: 30 Jul 2023
AbstractforPROSA—a multicenter prospective observational study to develop low-burden digital speech biomarkers in ALS and FTD | Full Text | References | PDF (773 KB) | EPUB
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Article
A speech-based prognostic model for dysarthria progression in ALS
Gabriela Stegmann, Sherman Charles, Julie Liss, Jeremy Shefner, Seward Rutkove & Visar Berisha
Pages: 599-604
Published online: 12 Jun 2023
AbstractforA speech-based prognostic model for dysarthria progression in ALS | Full Text | References | PDF (589.1 KB) | EPUB | Supplemental
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Clinical

Article
Development of ‘gastrostomy tube – is it for me?’, a web-based patient decision aid for people living with motor neurone disease considering having a gastrostomy tube placed
S. Wheelwright, R. Maunsell, S. Taylor, N. Drinkwater, C. Erridge, C. Foster, M. Hardcastle, A. Hogden, I. Lawson, D. Lisiecka, C. McDermott, K.E. Morrison, C. Muir, A. Recio-Saucedo & S. White
Pages: 605-613
Published online: 18 Jun 2023
AbstractforDevelopment of ‘gastrostomy tube – is it for me?’, a web-based patient decision aid for people living with motor neurone disease considering having a gastrostomy tube placed | Full Text | References | PDF (891.6 KB) | EPUB | Supplemental
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Open Access
Article
Demographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience
Inês Alves, Marta Gromicho, Miguel Oliveira Santos, Susana Pinto, Ana Pronto-Laborinho, Michael Swash & Mamede de Carvalho
Pages: 614-624
Published online: 09 Jun 2023
AbstractforDemographic changes in a large motor neuron disease cohort in Portugal: a 27 year experience | Full Text | References | PDF (1.2 MB) | EPUB | Supplemental
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Open Access
Article
The natural history of ALS: Baseline characteristics from a multicenter clinical cohort
Alex Berger, Matteo Locatelli, Ximena Arcila-Londono, Ghazala Hayat, Nicholas Olney, James Wymer, Kelly Gwathmey, Christian Lunetta, Terry Heiman-Patterson, Senda Ajroud-Driss, Eric A. Macklin, Marie-Abèle Bind, Kimberly Goslin, Tamela Stuchiner, Lauren Brown, Tracy Bazan, Tyler Regan, Ashley Adamo, Valerie Ferment, Carly Schroeder, Megan Somers, Georgios Manousakis, Kenneth Faulconer, Ervin Sinani, Julia Mirochnick, Hong Yu, Alexander V. Sherman, David Walk & The Pooled Resource Open-Access ALS Clinical Trials Consortium
Pages: 625-633
Published online: 17 Jul 2023
AbstractforThe natural history of ALS: Baseline characteristics from a multicenter clinical cohort | Full Text | References | PDF (2.1 MB) | EPUB | Supplemental
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Article
Crushing riluzole tablets: evaluation of loss of powder and active principle in a home-simulation experiment
Antonella Casiraghi, Andrea Gentile, Ivan Marjanovic & Adriano Chiò
Pages: 634-640
Published online: 16 Aug 2023
AbstractforCrushing riluzole tablets: evaluation of loss of powder and active principle in a home-simulation experiment | Full Text | References | PDF (737.9 KB) | EPUB | Supplemental
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Open Access
Article
IMPACT-ALS: summary of results from a European survey of people living with ALS
Eilis Conroy, Beatriz Vélez-Gómez, David O’Brien, Mark Heverin, Orla Hardiman, Christopher McDermott & Miriam Galvin
Pages: 641-650
Published online: 03 Sep 2023
AbstractforIMPACT-ALS: summary of results from a European survey of people living with ALS | Full Text | References | PDF (732.3 KB) | EPUB
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Article
Portable fixed dynamometry enables home-based, reliable assessment of muscle strength in patients with amyotrophic lateral sclerosis: a pilot study
Jordi W. J. Van Unnik, Jaap N.E. Bakers, Steure Kokx, Leonard H. Van Den Berg, Johanna M.A. Visser-Meily, Anita Beelen & Ruben P.A. Van Eijk
Pages: 651-660
Published online: 10 Jul 2023
AbstractforPortable fixed dynamometry enables home-based, reliable assessment of muscle strength in patients with amyotrophic lateral sclerosis: a pilot study | Full Text | References | PDF (960.2 KB) | EPUB | Supplemental
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Open Access
Article
Increased total daily energy expenditure in mild to moderate ALS: greater contribution from physical activity energy expenditure than hyper-metabolism
Rup Tandan, Diantha Howard & Dwight E. Matthews
Pages: 661-668
Published online: 03 Aug 2023
AbstractforIncreased total daily energy expenditure in mild to moderate ALS: greater contribution from physical activity energy expenditure than hyper-metabolism | Full Text | References | PDF (199.9 KB) | EPUB
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Epidemiology

Article
Analysis of the spatial distribution of amyotrophic lateral sclerosis in Virginia
Joseph Boyle, David C. Wheeler, Ryan Naum, Paula Burke Brockenbrough, Michelle Gebhardt, LaVon Smith, Tremetris Harrell, Danielle Stewart & Kelly Gwathmey
Pages: 669-677
Published online: 14 Jul 2023
AbstractforAnalysis of the spatial distribution of amyotrophic lateral sclerosis in Virginia | Full Text | References | PDF (578.4 KB) | EPUB | Supplemental
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Article
Epidemiological and clinical profile of amyotrophic lateral sclerosis in Ethiopia: a 5-year multicenter retrospective study
Dereje Melka, Hanna Demisse, Hanna Assefa, Yared Zenebe, Biniyam A. Ayele, Meron Awraris, Yohannese Gelan, Selam Kifelew, Medina Fedlu, Fikiru Tsehayneh, Mehila Zebenigus, Samson Alemayehu, Heera Tesfaye, Hildana Gulelat, Tsega Guta, Abenet Tafesse, Nebiyu Bekele, Marc Saez, Jan H. Veldink, Ammar Al-Chalabi, Monica Povedano & Ahmad Al Khleifat
Pages: 678-686
Published online: 26 Jul 2023
AbstractforEpidemiological and clinical profile of amyotrophic lateral sclerosis in Ethiopia: a 5-year multicenter retrospective study | Full Text | References | PDF (598.4 KB) | EPUB | Supplemental
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Article
Comparing Amyotrophic lateral sclerosis (ALS) patient characteristics from the National ALS Registry and the Massachusetts ALS Registry, data through 2015
Jaime Raymond, Reshma Punjani, Theodore Larson, James D. Berry, D. Kevin Horton & Paul Mehta
Pages: 687-694
Published online: 04 Aug 2023
AbstractforComparing Amyotrophic lateral sclerosis (ALS) patient characteristics from the National ALS Registry and the Massachusetts ALS Registry, data through 2015 | Full Text | References | PDF (200.5 KB) | EPUB
  • 562 Views
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Open Access
Article
An electronic health record cohort of Veterans with amyotrophic lateral sclerosis
Richard J. Reimer, Andre Goncalves, Braden Soper, Jose Cadena, Jennifer L. Wilson, Amy L. Gryshuk, Paola Suarez, Thomas F. Osborne, Kevin V. Grimes & Priyadip Ray
Pages: 695-701
Published online: 09 Aug 2023
AbstractforAn electronic health record cohort of Veterans with amyotrophic lateral sclerosis | Full Text | References | PDF (334.1 KB) | EPUB
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Open Access
Article
Prevalence of amyotrophic lateral sclerosis in the United States, 2018
Paul Mehta, Jaime Raymond, Yuzi Zhang, Reshma Punjani, Moon Han, Theodore Larson, Oleg Muravov, Robert H. Lyles & D. Kevin Horton
Pages: 702-708
Published online: 21 Aug 2023
AbstractforPrevalence of amyotrophic lateral sclerosis in the United States, 2018 | Full Text | References | PDF (538.2 KB) | EPUB
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Open Access

Cognition

Brief Report
Single task-level, 2SD-based cutoffs for the Italian version of the Edinburgh Cognitive and Behavioral ALS screen (ECAS)
Barbara Poletti, Edoardo Nicolò Aiello, Antonino La Tona, Federica Solca, Silvia Torre, Eleonora Colombo, Alessio Maranzano, Claudia Morelli, Alberto Doretti, Federico Verde, Alessia Monti, Agostino Brugnera, Angelo Compare, Roberta Ferrucci, Sergio Barbieri, Francesca Mameli, Alberto Priori, Gabriella Pravettoni, Vincenzo Silani & Nicola Ticozzi
Pages: 709-712
Published online: 08 Jun 2023
AbstractforSingle task-level, 2SD-based cutoffs for the Italian version of the Edinburgh Cognitive and Behavioral ALS screen (ECAS) | Full Text | References | PDF (579.2 KB) | EPUB | Supplemental
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Article
Validity and reliability measures of the Swedish Karolinska version of the Edinburgh Cognitive and Behavioral ALS Screen (SK-ECAS)
Juliette Foucher, Ivar Winroth, Anikó Lovik, Stefan Sennfält, Joana B. Pereira, Fang Fang, Dorothee Lule, Peter M. Andersen & Caroline Ingre
Pages: 713-718
Published online: 31 Jul 2023
AbstractforValidity and reliability measures of the Swedish Karolinska version of the Edinburgh Cognitive and Behavioral ALS Screen (SK-ECAS) | Full Text | References | PDF (581.3 KB) | EPUB | Supplemental
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Open Access

Caregiver Burden

Review Article
Resilient together-ALS: leveraging the NDD transdiagnostic framework to develop an early dyadic intervention for people with amyotrophic lateral sclerosis and their informal care-partners
Christina L. Rush, Ethan G. Lester, Heena Manglani, Emily Woodworth, Ottavio Vitolo, Maurizio Fava, James D. Berry, Kate Brizzi, Suma Babu, Elizabeth C. Lindenberger, J. Randall Curtis & Ana-Maria Vranceanu
Pages: 719-726
Published online: 22 Jun 2023
AbstractforResilient together-ALS: leveraging the NDD transdiagnostic framework to develop an early dyadic intervention for people with amyotrophic lateral sclerosis and their informal care-partners | Full Text | References | PDF (413.7 KB) | EPUB
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Article
Living with a parent with ALS - adolescents’ need for professional support from the adolescents’ and the parents’ perspectives
Nina Malmström, Birgitta Jakobsson Larsson, Stefan Nilsson, Joakim Öhlén, Ingela Nygren, Peter M. Andersen & Anneli Ozanne
Pages: 727-735
Published online: 24 Jul 2023
AbstractforLiving with a parent with ALS - adolescents’ need for professional support from the adolescents’ and the parents’ perspectives | Full Text | References | PDF (279.2 KB) | EPUB
  • 731 Views
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Open Access

Genomics

Article
SOD1-ALS-Browser: a web-utility for investigating the clinical phenotype in SOD1 amyotrophic lateral sclerosis
Thomas P Spargo, Sarah Opie-Martin, Guy P Hunt, Munishikha Kalia, Ahmad Al Khleifat, Simon D Topp, Christopher E Shaw, Ammar Al-Chalabi, Alfredo Iacoangeli & The Project Mine ALS Sequencing Consortium
Pages: 736-745
Published online: 03 Aug 2023
AbstractforSOD1-ALS-Browser: a web-utility for investigating the clinical phenotype in SOD1 amyotrophic lateral sclerosis | Full Text | References | PDF (1,005.6 KB) | EPUB | Supplemental
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Open Access

Animal Models

Article
Aberrant dynein function promotes TDP-43 aggregation and upregulation of p62 in male mice harboring transgenic human TDP-43
Eleni Christoforidou, Fabio A. Simoes, David Gordon, Kevin Talbot & Majid Hafezparast
Pages: 746-755
Published online: 27 Jul 2023
AbstractforAberrant dynein function promotes TDP-43 aggregation and upregulation of p62 in male mice harboring transgenic human TDP-43 | Full Text | References | PDF (2.1 MB) | EPUB
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Open Access
Article
The cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1G93A mice
Linda Greensmith & J. Barney Bryson
Pages: 756-762
Published online: 25 Jul 2023
AbstractforThe cholesterol depleting agent, (2-Hydroxypropyl)-ß-cyclodextrin, does not affect disease progression in SOD1G93A mice | Full Text | References | PDF (616.2 KB) | EPUB
  • 736 Views
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Open Access

Brief Report

Brief Report
Slowly progressive late-onset spinal muscular atrophy Finkel-type related to p.Pro56Ser VABP mutation in Colombia
Cristian Correa-Arrieta, Fernando Ortiz-Corredor, Sandra Castellar-Leones & Diana Sánchez-Peñarete
Pages: 763-765
Published online: 06 Aug 2023
AbstractforSlowly progressive late-onset spinal muscular atrophy Finkel-type related to p.Pro56Ser VABP mutation in Colombia | Full Text | References | PDF (649 KB) | EPUB
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ALS Untangled

Case Report
ALSUntangled #70: caffeine
Jessica Hatch, Paul Barkhaus, Benjamin Barnes, Morgan Beauchamp, Michael Benatar, Tulio Bertorini, Robert Bowser, Mark Bromberg, Andrew Brown, Javier Mascias Cadavid, Gregory T. Carter, Nicholas Cole, Jesse Crayle, Mazen Dimachkie, David Ennist, Eva Feldman, Timothy Fullam, Terry Heiman-Patterson, Sartaj Jhooty, Todd Levine, Xiaoyan Li, Isaac Lund, Elise Mallon, Nicholas Maragakis, Christopher McDermott, Gary Pattee, Kaitlyn Pierce, Dylan Ratner, Kim Staats, Paul Wicks, Martina Wiedau & Richard Bedlack
Pages: 766-770
Published online: 08 Jun 2023
AbstractforALSUntangled #70: caffeine | Full Text | References | PDF (347 KB) | EPUB
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Free Access

Correction

Correction
Correction
Page: 771
Published online: 03 Aug 2023
CitationforCorrection | Full Text | PDF (93.9 KB) | EPUB
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Free Access

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