Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Volume 15, Issue 5-6 (2014)

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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Volume 15, Issue 5-6 (2014)

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Review Articles

Review Article
RNA metabolism in ALS: When normal processes become pathological
Cristian A. Droppelmann, Danae Campos-Melo, Muhammad Ishtiaq, Kathryn Volkening & Michael J. Strong
Pages: 321-336
Published online: 20 Feb 2014
AbstractforRNA metabolism in ALS: When normal processes become pathological | Full Text | References | PDF (1 MB)
  • 1426 Views
  • 43 CrossRef citations
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Review Article

Review Article
Assessment of disease progression and functional benefit in neurodegenerative disease: Can we tell the difference?
Jeremy M. Shefner & Dragos Mihaila
Pages: 337-343
Published online: 11 Jun 2014
AbstractforAssessment of disease progression and functional benefit in neurodegenerative disease: Can we tell the difference? | Full Text | References | PDF (71.8 KB)
  • 222 Views
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Original Articles: Biomarkers

Article
Multicentre quality control evaluation of different biomarker candidates for amyotrophic lateral sclerosis
Stefan Lehnert, Julia Costa, Mamede de Carvalho, Janine Kirby, Magdalena Kuzma-Kozakiewicz, Claudia Morelli, Wim Robberecht, Pamela Shaw, Vincenzo Silani, Petra Steinacker, Hayrettin Tumani, Philip Van Damme, Albert Ludolph & Markus Otto
Pages: 344-350
Published online: 28 Feb 2014
AbstractforMulticentre quality control evaluation of different biomarker candidates for amyotrophic lateral sclerosis | Full Text | References | PDF (545.4 KB)
  • 660 Views
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Research Article

Article
Limited role of free TDP-43 as a diagnostic tool in neurodegenerative diseases
Emily Feneberg, Petra Steinacker, Stefan Lehnert, Anja Schneider, Paul Walther, Dietmar R. Thal, Miriam Linsenmeier, Albert C. Ludolph & Markus Otto
Pages: 351-356
Published online: 16 May 2014
AbstractforLimited role of free TDP-43 as a diagnostic tool in neurodegenerative diseases | Full Text | References | PDF (1.2 MB) | Supplemental
  • 1637 Views
  • 107 CrossRef citations
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Article
Increased iron level in motor cortex of amyotrophic lateral sclerosis patients: An in vivo MR study
Jixu Yu, Faying Qi, Nian Wang, Peihong Gao, Shouping Dai, Yucheng Lu, Quanping Su, Yifeng Du & Fengyuan Che
Pages: 357-361
Published online: 09 May 2014
AbstractforIncreased iron level in motor cortex of amyotrophic lateral sclerosis patients: An in vivo MR study | Full Text | References | PDF (478.8 KB)
  • 311 Views
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Article
Plasma metabolomic biomarker panel to distinguish patients with amyotrophic lateral sclerosis from disease mimics
Kay A. Lawton, Meredith V. Brown, Danny Alexander, Zhen Li, Jacob E. Wulff, Robert Lawson, Matt Jaffa, Mike V. Milburn, John A. Ryals, Robert Bowser, Merit E. Cudkowicz, James D. Berry & On behalf of the Northeast ALS Consortium
Pages: 362-370
Published online: 01 Jul 2014
AbstractforPlasma metabolomic biomarker panel to distinguish patients with amyotrophic lateral sclerosis from disease mimics | Full Text | References | PDF (1,006.5 KB) | Supplemental
  • 967 Views
  • 65 CrossRef citations
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Article
Plasma amino acids patterns and age of onset of amyotrophic lateral sclerosis
Matteo Cecchi, Paolo Messina, Luisa Airoldi, Elisabetta Pupillo, Monica Bandettini di Poggio, Andrea Calvo, Massimiliano Filosto, Christian Lunetta, Jessica Mandrioli, Federica Pisa, Roberta Pastorelli, Ettore Beghi & Eurals Consortium
Pages: 371-375
Published online: 06 Jun 2014
AbstractforPlasma amino acids patterns and age of onset of amyotrophic lateral sclerosis | Full Text | References | PDF (99.6 KB) | Supplemental
  • 198 Views
  • 8 CrossRef citations
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Cognition, Neuropsychology

Article
Psychological as well as illness factors influence acceptance of non-invasive ventilation (NIV) and gastrostomy in amyotrophic lateral sclerosis (ALS): A prospective population study
Naomi H. Martin, Sabine Landau, Anna Janssen, Rebecca Lyall, Irene Higginson, Rachel Burman, Paul McCrone, Mohammed Sakel, Catherine M. Ellis, Christopher E. Shaw, Ammar Al-Chalabi, P. Nigel Leigh & Laura H. Goldstein
Pages: 376-387
Published online: 06 Mar 2014
AbstractforPsychological as well as illness factors influence acceptance of non-invasive ventilation (NIV) and gastrostomy in amyotrophic lateral sclerosis (ALS): A prospective population study | Full Text | References | PDF (289.9 KB)
  • 773 Views
  • 36 CrossRef citations
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Research Article

Article
The verbal fluency index: Dutch normative data for cognitive testing in ALS
Emma Beeldman, Bregje Jaeger, Joost Raaphorst, Meinie Seelen, Jan Veldink, Leonard van den Berg, Marianne de Visser & Ben Schmand
Pages: 388-391
Published online: 27 May 2014
AbstractforThe verbal fluency index: Dutch normative data for cognitive testing in ALS | Full Text | References | PDF (56.3 KB)
  • 523 Views
  • 13 CrossRef citations
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Article
The impact of physical impairment on emotional well-being in ALS
Susanne Abdulla, Stefan Vielhaber, Katja Kollewe, Judith Machts, Hans-Jochen Heinze, Reinhard Dengler & Susanne Petri
Pages: 392-397
Published online: 10 Jul 2014
AbstractforThe impact of physical impairment on emotional well-being in ALS | Full Text | References | PDF (184.5 KB)
  • 467 Views
  • 14 CrossRef citations
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Clinical Trials

Article
Seeking a measure of clinically meaningful change in ALS
Martin McElhiney, Judith G. Rabkin, Raymond Goetz, Jonathan Katz, Robert G. Miller, Dallas A. Forshew, William David, Merit Cudkowicz, Jonathan D. Glass, Stanley Appel, Ericka Simpson & Hiroshi Mitsumoto
Pages: 398-405
Published online: 15 Aug 2014
AbstractforSeeking a measure of clinically meaningful change in ALS | Full Text | References | PDF (84.2 KB)
  • 494 Views
  • 11 CrossRef citations
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Research Article

Article
A post hoc analysis of subgroup outcomes and creatinine in the phase III clinical trial (EMPOWER) of dexpramipexole in ALS
Michael E. Bozik, Hiroshi Mitsumoto, Benjamin R. Brooks, Stacy A. Rudnicki, Dan H. Moore, Bing Zhang, Albert Ludolph, Merit E. Cudkowicz, Leonard H. van den Berg, James Mather, Thomas Petzinger Jr & Donald Archibald
Pages: 406-413
Published online: 15 Aug 2014
AbstractforA post hoc analysis of subgroup outcomes and creatinine in the phase III clinical trial (EMPOWER) of dexpramipexole in ALS | Full Text | References | PDF (210.3 KB)
  • 615 Views
  • 45 CrossRef citations
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Health Services & Health Economics

Article
Diagnostic yield and cost-effectiveness of investigations in patients presenting with isolated lower motor neuron signs
Mohammed H. Alanazy, Chris White & Lawrence Korngut
Pages: 414-419
Published online: 27 May 2014
AbstractforDiagnostic yield and cost-effectiveness of investigations in patients presenting with isolated lower motor neuron signs | Full Text | References | PDF (65.2 KB)
  • 201 Views
  • 4 CrossRef citations
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Research Article

Article
The provision of assistive devices and home adaptations to patients with ALS in the Netherlands: Patients’ perspectives
Huub Creemers, Anita Beelen, Hepke Grupstra, Frans Nollet & Leonard H. van den Berg
Pages: 420-425
Published online: 05 Jun 2014
AbstractforThe provision of assistive devices and home adaptations to patients with ALS in the Netherlands: Patients’ perspectives | Full Text | References | PDF (147.2 KB)
  • 616 Views
  • 16 CrossRef citations
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Article
Economic burden of amyotrophic lateral sclerosis: A Canadian study of out-of-pocket expenses
Matthew Gladman, Celina Dharamshi & Lorne Zinman
Pages: 426-432
Published online: 15 Jul 2014
AbstractforEconomic burden of amyotrophic lateral sclerosis: A Canadian study of out-of-pocket expenses | Full Text | References | PDF (79.4 KB)
  • 800 Views
  • 35 CrossRef citations
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Epidemiology, Phenotyping

Article
Feasibility of creating a National ALS Registry using administrative data in the United States
Wendy E. Kaye, Marchelle Sanchez & Jennifer Wu
Pages: 433-439
Published online: 06 Mar 2014
AbstractforFeasibility of creating a National ALS Registry using administrative data in the United States | Full Text | References | PDF (79.5 KB)
  • 310 Views
  • 28 CrossRef citations
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Research Article

Article
Minorities, men, and unmarried amyotrophic lateral sclerosis patients are more likely to die in an acute care facility
Stephen A. Goutman, Dustin G. Nowacek, James F. Burke, Kevin A. Kerber, Lesli E. Skolarus & Brian C. Callaghan
Pages: 440-443
Published online: 12 Jun 2014
AbstractforMinorities, men, and unmarried amyotrophic lateral sclerosis patients are more likely to die in an acute care facility | Full Text | References | PDF (57.4 KB)
  • 209 Views
  • 15 CrossRef citations
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Article
RandomForest4Life: A Random Forest for predicting ALS disease progression
Torsten Hothorn & Hans H. Jung
Pages: 444-452
Published online: 20 Aug 2014
AbstractforRandomForest4Life: A Random Forest for predicting ALS disease progression | Full Text | References | PDF (1 MB)
  • 791 Views
  • 48 CrossRef citations
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Article
Diagnostic timelines and delays in diagnosing amyotrophic lateral sclerosis (ALS)
Sabrina Paganoni, Eric A. Macklin, Alexandra Lee, Alyssa Murphy, Judith Chang, Amanda Zipf, Merit Cudkowicz & Nazem Atassi
Pages: 453-456
Published online: 01 Jul 2014
AbstractforDiagnostic timelines and delays in diagnosing amyotrophic lateral sclerosis (ALS) | Full Text | References | PDF (141.5 KB)
  • 960 Views
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Review Article

Review Article
Monomelic amyotrophy: Clinical profile and natural history of 279 cases seen over 35 years (1976–2010)
Atchayaram Nalini, Mandavilli Gourie-Devi, Kandavel Thennarasu & Aravinda Hanumanthapura Ramalingaiah
Pages: 457-465
Published online: 22 May 2014
AbstractforMonomelic amyotrophy: Clinical profile and natural history of 279 cases seen over 35 years (1976–2010) | Full Text | References | PDF (408.7 KB)
  • 746 Views
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Short Report: Pathology

Brief Report
Atypical Alzheimer's disease in an elderly United States resident with amyotrophic lateral sclerosis and pathological tau in spinal motor neurons
Leo F. McCluskey, Felix Geser, Lauren B. Elman, Vivianna M. Van Deerlin, John L. Robinson, Virginia M.-Y. Lee & John Q. Trojanowski
Pages: 466-472
Published online: 09 May 2014
First Page PreviewforAtypical Alzheimer's disease in an elderly United States resident with amyotrophic lateral sclerosis and pathological tau in spinal motor neurons | Full Text | References | PDF (919.2 KB)
  • 109 Views
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Obituary

Obituary
Theodore Leon ‘Ted’ Munsat MD (1930–2013)
Benjamin Rix Brooks & Michael Swash
Pages: 473-474
Published online: 09 May 2014
CitationforTheodore Leon ‘Ted’ Munsat MD (1930–2013) | Full Text | References | PDF (550.3 KB)
  • 397 Views
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Free Access

ALS-UNTANGLED

Article
ALSUntangled No. 25: Ursodiol
The ALSUntangled Group
Pages: 475-478
Published online: 27 Jun 2014
First Page PreviewforALSUntangled No. 25: Ursodiol | Full Text | References | PDF (55.8 KB)
  • 3908 Views
  • 1 CrossRef citations
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Free Access

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