Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Volume 18, Issue 3-4 (2017)

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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Volume 18, Issue 3-4 (2017)

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Research Article

Article
Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria
Michael J. Strong, Sharon Abrahams, Laura H. Goldstein, Susan Woolley, Paula Mclaughlin, Julie Snowden, Eneida Mioshi, Angie Roberts-South, Michael Benatar, Tibor HortobáGyi, Jeffrey Rosenfeld, Vincenzo Silani, Paul G Ince & Martin R. Turner
Pages: 153-174
Published online: 05 Jan 2017
AbstractforAmyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria | Full Text | References | PDF (620.4 KB) | Supplemental
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Original Article

Article
Proposed association between the hexanucleotide repeat of C9orf72 and opposability index of the thumb
Zhongbo Chen, Kuang Lin, Jeffrey D. Macklis & Ammar Al-Chalabi
Pages: 175-181
Published online: 23 Dec 2016
AbstractforProposed association between the hexanucleotide repeat of C9orf72 and opposability index of the thumb | Full Text | References | PDF (585.2 KB) | Supplemental
  • 1506 Views
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Open Access
Article
Assessing cognitive functioning in ALS: A focus on frontal lobe processes
S. M. Gillingham, Y. Yunusova, A. Ganda, E. Rogaeva, S. E. Black, D. T. Stuss & L. Zinman
Pages: 182-192
Published online: 08 Dec 2016
AbstractforAssessing cognitive functioning in ALS: A focus on frontal lobe processes | Full Text | References | PDF (691.6 KB)
  • 594 Views
  • 22 CrossRef citations
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Research Article

Article
Visual encoding, consolidation, and retrieval in amyotrophic lateral sclerosis: executive function as a mediator, and predictor of performance
Tom Burke, Katie Lonergan, Marta Pinto-Grau, Marwa Elamin, Peter Bede, Caoifa Madden, Orla Hardiman & Niall Pender
Pages: 193-201
Published online: 13 Jan 2017
AbstractforVisual encoding, consolidation, and retrieval in amyotrophic lateral sclerosis: executive function as a mediator, and predictor of performance | Full Text | References | PDF (697.5 KB)
  • 300 Views
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Original Article

Article
Longitudinal assessment of the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS): lack of practice effect in ALS patients?
Christian Burkhardt, Christoph Neuwirth & Markus Weber
Pages: 202-209
Published online: 06 Feb 2017
AbstractforLongitudinal assessment of the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS): lack of practice effect in ALS patients? | Full Text | References | PDF (639.6 KB) | Supplemental
  • 475 Views
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Research Article

Article
Proteomic profiling of the spinal cord in ALS: decreased ATP5D levels suggest synaptic dysfunction in ALS pathogenesis
Jooyeon Engelen-Lee, Anna M. Blokhuis, Wim G. M. Spliet, R. Jeroen Pasterkamp, Eleonora Aronica, Jeroen A. A. Demmers, Roel Broekhuizen, Giovanni Nardo, Niels Bovenschen & Leonard H. Van Den Berg
Pages: 210-220
Published online: 29 Nov 2016
AbstractforProteomic profiling of the spinal cord in ALS: decreased ATP5D levels suggest synaptic dysfunction in ALS pathogenesis | Full Text | References | PDF (2.3 MB)
  • 2932 Views
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Open Access
Article
Is firstly diagnosed ALS really ALS? Results of a population-based study with long-term follow-up
Elisabetta Pupillo, Elisa Bianchi, Marco Poloni, Ettore Beghi & for the SLALOM Group
Pages: 221-226
Published online: 28 Nov 2016
AbstractforIs firstly diagnosed ALS really ALS? Results of a population-based study with long-term follow-up | Full Text | References | PDF (264 KB)
  • 312 Views
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Original Article

Article
Comparison of the King’s and MiToS staging systems for ALS
Ton Fang, Ahmad Al Khleifat, Daniel R Stahl, Claudia Lazo La Torre, Caroline Murphy, Uk-Mnd LicalS, Carolyn Young, Pamela J Shaw, P Nigel Leigh & Ammar Al-Chalabi
Pages: 227-232
Published online: 05 Jan 2017
AbstractforComparison of the King’s and MiToS staging systems for ALS | Full Text | References | PDF (719.8 KB)
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Open Access

Research Article

Article
Percutaneous endoscopic gastrostomy, body weight loss and survival in amyotrophic lateral sclerosis: a population-based registry study
Antonio Fasano, Nicola Fini, Diana Ferraro, Laura Ferri, Marco Vinceti, Errals & Jessica Mandrioli
Pages: 233-242
Published online: 11 Jan 2017
AbstractforPercutaneous endoscopic gastrostomy, body weight loss and survival in amyotrophic lateral sclerosis: a population-based registry study | Full Text | References | PDF (498 KB)
  • 898 Views
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Article
A risk stratifying tool to facilitate safe late-stage percutaneous endoscopic gastrostomy in ALS
Alexander G. Thompson, Victoria Blackwell, Rachael Marsden, Emma Millard, Clare Lawson, Annabel H. Nickol, James E. East, Kevin Talbot, Philip J. Allan & Martin R. Turner
Pages: 243-248
Published online: 19 Jan 2017
AbstractforA risk stratifying tool to facilitate safe late-stage percutaneous endoscopic gastrostomy in ALS | Full Text | References | PDF (809.2 KB)
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Open Access
Article
Genetic analysis of patients with familial and sporadic amyotrophic lateral sclerosis in a Brazilian Research Center
Gerson Chadi, Jessica Ruivo Maximino, Frederico Mennucci de Haidar Jorge, Fabrício Castro de Borba, Joyce Meire Gilio, Dagoberto Callegaro, Camila Galvão Lopes, Samantha Nakamura Dos Santos & Gabriela Natania Sales Rebelo
Pages: 249-255
Published online: 15 Dec 2016
AbstractforGenetic analysis of patients with familial and sporadic amyotrophic lateral sclerosis in a Brazilian Research Center | Full Text | References | PDF (142.9 KB) | Supplemental
  • 522 Views
  • 28 CrossRef citations
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Article
Sequence variations in C9orf72 downstream of the hexanucleotide repeat region and its effect on repeat-primed PCR interpretation: a large multinational screening study
Angelica Nordin, Chizuru Akimoto, Anna Wuolikainen, Helena Alstermark, Karin Forsberg, Peter Baumann, Susana Pinto, Mamede de Carvalho, Annemarie Hübers, Frida Nordin, Albert C. Ludolph, Jochen H. Weishaupt, Thomas Meyer, Torsten Grehl, Kathi Schweikert, Markus Weber, Christian Burkhardt, Christoph Neuwirth, Trygve Holmøy, Mitsuya Morita, Ole-Bjørn Tysnes, Michael Benatar, Joanne Wuu, Dale J. Lange, Carsten Bisgård, Nasrin Asgari, Ilkka Tarvainen, Thomas Brännström & Peter M. Andersen
Pages: 256-264
Published online: 12 Dec 2016
AbstractforSequence variations in C9orf72 downstream of the hexanucleotide repeat region and its effect on repeat-primed PCR interpretation: a large multinational screening study | Full Text | References | PDF (1.8 MB)
  • 566 Views
  • 15 CrossRef citations
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Article
Mutations of CCNF gene is rare in patients with amyotrophic lateral sclerosis and frontotemporal dementia from Mainland China
Chuzheng Pan, Bin Jiao, Tingting Xiao, Lihua Hou, Weiwei Zhang, Xi Liu, Jun Xu, Beisha Tang & Lu Shen
Pages: 265-268
Published online: 10 Mar 2017
AbstractforMutations of CCNF gene is rare in patients with amyotrophic lateral sclerosis and frontotemporal dementia from Mainland China | Full Text | References | PDF (296 KB)
  • 449 Views
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Article
Open label study to assess the safety of VM202 in subjects with amyotrophic lateral sclerosis
Robert L. Sufit, Senda Ajroud-Driss, Patricia Casey & John A. Kessler
Pages: 269-278
Published online: 06 Feb 2017
AbstractforOpen label study to assess the safety of VM202 in subjects with amyotrophic lateral sclerosis | Full Text | References | PDF (565.8 KB)
  • 1013 Views
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Original Article

Article
Geographic distributions of motor neuron disease mortality and well water use in U.S. counties
Gary G. Schwartz, Bradley C. Rundquist, Isaac J. Simon & Sami E. Swartz
Pages: 279-283
Published online: 25 Dec 2016
AbstractforGeographic distributions of motor neuron disease mortality and well water use in U.S. counties | Full Text | References | PDF (883.5 KB)
  • 3857 Views
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Open Access
Article
Incidence of amyotrophic lateral sclerosis in the province of Novara, Italy, and possible role of environmental pollution
Marina Tesauro, Michela Consonni, Tommaso Filippini, Letizia Mazzini, Fabrizio Pisano, Adriano Chiò, Aniello Esposito & Marco Vinceti
Pages: 284-290
Published online: 02 Feb 2017
AbstractforIncidence of amyotrophic lateral sclerosis in the province of Novara, Italy, and possible role of environmental pollution | Full Text | References | PDF (388.2 KB)
  • 305 Views
  • 19 CrossRef citations
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Article
Seasons and ALS time of death
Susana Pinto & Mamede De Carvalho
Pages: 291-295
Published online: 10 Mar 2017
AbstractforSeasons and ALS time of death | Full Text | References | PDF (246.2 KB)
  • 215 Views
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Report

Report
Slowly progressive motor neuron disease with multi-system involvement related to p.E121G SOD1 mutation
Guillaume Taieb, Anne Polge, Raul Juntas-Morales, Nicolas Pageot, Serge Lumbroso, Kevin Mouzat & William Camu
Pages: 296-297
Published online: 28 Nov 2016
AbstractforSlowly progressive motor neuron disease with multi-system involvement related to p.E121G SOD1 mutation | Full Text | References | PDF (97.5 KB)
  • 123 Views
  • 1 CrossRef citations
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Short Report

Report
A novel mutation of the C-terminal amino acid of FUS (Y526C) strengthens FUS gene as the most frequent genetic factor in aggressive juvenile ALS
Philippe Corcia, Veronique Danel, Arnaud Lacour, Stephane Beltran, Christian Andres, Philippe Couratier, Helene Blasco & Patrick Vourc’h
Pages: 298-301
Published online: 05 Jan 2017
AbstractforA novel mutation of the C-terminal amino acid of FUS (Y526C) strengthens FUS gene as the most frequent genetic factor in aggressive juvenile ALS | Full Text | References | PDF (348.7 KB)
  • 359 Views
  • 16 CrossRef citations
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Report
Further analysis of KIFAP3 gene in ALS patients from Switzerland and Sweden
David Czell, Peter C. Sapp, Christoph Neuwirth, Markus Weber, Peter M. Andersen & Robert H. Brown
Pages: 302-304
Published online: 31 Jan 2017
AbstractforFurther analysis of KIFAP3 gene in ALS patients from Switzerland and Sweden | Full Text | References | PDF (385.6 KB)
  • 262 Views
  • 4 CrossRef citations
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Letter to the Editor

Letter
Survival in amyotrophic lateral sclerosis patients on non-invasive ventilation. What can we do more?
Antonio M. Esquinas, Giancarlo Garuti, Giulia Michela Pellegrino & Giuseppe Francesco Sferrazza Papa
Pages: 305-306
Published online: 15 Sep 2016
CitationforSurvival in amyotrophic lateral sclerosis patients on non-invasive ventilation. What can we do more? | Full Text | References | PDF (85.2 KB)
  • 204 Views
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Letter
Initiation of Non-invasive ventilation in amyotrophic lateral sclerosis: looking for some details from the guidelines
Giuseppe Fiorentino & Antonio M. Esquinas
Pages: 307-308
Published online: 13 Mar 2017
CitationforInitiation of Non-invasive ventilation in amyotrophic lateral sclerosis: looking for some details from the guidelines | Full Text | References | PDF (193.2 KB)
  • 223 Views
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Original Articles

Article
ALSUntangled No. 37: Inosine*
The ALSUntangled Group
Pages: 309-312
Published online: 30 Aug 2016
First Page PreviewforALSUntangled No. 37: Inosine* | Full Text | References | PDF (123.8 KB)
  • 3285 Views
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