Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Volume 20, Issue 3-4 (2019)

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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, Volume 20, Issue 3-4 (2019)

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Review Article

Review Article
Primary lateral sclerosis: a distinct entity or part of the ALS spectrum?
Eoin Finegan, Rangariroyashe H. Chipika, Stacey Li Hi Shing, Orla Hardiman & Peter Bede
Pages: 133-145
Published online: 18 Jan 2019
AbstractforPrimary lateral sclerosis: a distinct entity or part of the ALS spectrum? | Full Text | References | PDF (184 KB) | EPUB
  • 932 Views
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Research Article

Article
Primary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates
Giulia Vinceti, Nicholas Olney, Maria Luisa Mandelli, Salvatore Spina, H. Isabel Hubbard, Miguel A. Santos-Santos, Christa Watson, Zachary A. Miller, Catherine Lomen-Hoerth, Paolo Nichelli, Bruce L. Miller, Lea T. Grinberg, William W. Seeley & Maria Luisa Gorno-Tempini
Pages: 146-158
Published online: 22 Jan 2019
AbstractforPrimary progressive aphasia and the FTD-MND spectrum disorders: clinical, pathological, and neuroimaging correlates | Full Text | References | PDF (695.6 KB) | EPUB | Supplemental
  • 1105 Views
  • 19 CrossRef citations
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Article
A standard operating procedure for King’s ALS clinical staging
Rubika Balendra, Ahmad Al Khleifat, Ton Fang & Ammar Al-Chalabi
Pages: 159-164
Published online: 18 Feb 2019
AbstractforA standard operating procedure for King’s ALS clinical staging | Full Text | References | PDF (502 KB) | EPUB | Supplemental
  • 4738 Views
  • 21 CrossRef citations
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Open Access
Article
Improved survival and 30-day mortality after gastrostomy in Scottish motor neurone disease patients: evidence from a national retrospective cohort study using STROBE criteria
George H. Gorrie, Siddharthan Chandran, Shuna Colville, Judith Newton, Danielle Leighton, Menai Mcdonald, Suvankar Pal, Raeburn Forbes, Mario Hair & Robert Swingler
Pages: 165-171
Published online: 05 Mar 2019
AbstractforImproved survival and 30-day mortality after gastrostomy in Scottish motor neurone disease patients: evidence from a national retrospective cohort study using STROBE criteria | Full Text | References | PDF (186.7 KB) | EPUB
  • 466 Views
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Article
Facial onset sensory and motor neuronopathy: a motor neuron disease with an oligogenic origin?
Juan Francisco Vázquez-Costa, Laia Pedrola Vidal, Sarah Moreau-Le Lan, Irene Teresí-Copoví, Marina Frasquet, Maria J. Chumillas & Teresa Sevilla
Pages: 172-175
Published online: 20 Mar 2019
AbstractforFacial onset sensory and motor neuronopathy: a motor neuron disease with an oligogenic origin? | Full Text | References | PDF (315.9 KB) | EPUB
  • 397 Views
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Original Article

Article
Predictors of diagnostic delay in amyotrophic lateral sclerosis: a cohort study based on administrative and electronic medical records data
Francesca Palese, Arianna Sartori, Giancarlo Logroscino & Federica Edith Pisa
Pages: 176-185
Published online: 18 Jan 2019
AbstractforPredictors of diagnostic delay in amyotrophic lateral sclerosis: a cohort study based on administrative and electronic medical records data | Full Text | References | PDF (702.1 KB) | EPUB | Supplemental
  • 425 Views
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Research Article

Article
Elevated serum ferritin level as a predictor of reduced survival in patients with sporadic amyotrophic lateral sclerosis in China: a retrospective study
Qionghua Sun, Fei Yang, Hongfen Wang, Fang Cui, Yanran Li, Siyuan Li, Yuting Ren, Wenjie Lan, Mao Li, Wenjia Zhu, Xiaosun Sun, Zhengqing He, Xue Zhao, Li Yang & Xusheng Huang
Pages: 186-191
Published online: 17 Jan 2019
AbstractforElevated serum ferritin level as a predictor of reduced survival in patients with sporadic amyotrophic lateral sclerosis in China: a retrospective study | Full Text | References | PDF (300.5 KB) | EPUB
  • 173 Views
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Article
Excitability in somatosensory cortex correlates with motoric impairment in amyotrophic lateral sclerosis
Oliver Höffken, Alena Schmelz, Melanie Lenz, Kai Gruhn, Torsten Grehl, Martin Tegenthoff & Matthias Sczesny-Kaiser
Pages: 192-198
Published online: 23 Feb 2019
AbstractforExcitability in somatosensory cortex correlates with motoric impairment in amyotrophic lateral sclerosis | Full Text | References | PDF (433.6 KB) | EPUB
  • 271 Views
  • 9 CrossRef citations
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Article
Plasma creatinine and amyotrophic lateral sclerosis prognosis: a systematic review and meta-analysis
Débora Lanznaster, Theodora Bejan-Angoulvant, Franck Patin, Christian R. Andres, Patrick Vourc’h, Phillipe Corcia & HÉlÉne Blasco
Pages: 199-206
Published online: 09 Apr 2019
AbstractforPlasma creatinine and amyotrophic lateral sclerosis prognosis: a systematic review and meta-analysis | Full Text | References | PDF (1.1 MB) | EPUB | Supplemental
  • 445 Views
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Article
ALSgeneScanner: a pipeline for the analysis and interpretation of DNA sequencing data of ALS patients
Alfredo Iacoangeli, Ahmad Al Khleifat, William Sproviero, Aleksey Shatunov, Ashley R. Jones, Sarah Opie-Martin, Ersilia Naselli, Simon D. Topp, Isabella Fogh, Angela Hodges, Richard J. Dobson, Stephen J. Newhouse & Ammar Al-Chalabi
Pages: 207-215
Published online: 05 Mar 2019
AbstractforALSgeneScanner: a pipeline for the analysis and interpretation of DNA sequencing data of ALS patients | Full Text | References | PDF (839.6 KB) | EPUB | Supplemental
  • 3334 Views
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Open Access

Original Articles

Article
Lack of consensus in ALS genetic testing practices and divergent views between ALS clinicians and patients
Holly Klepek, Haikady Nagaraja, Stephen A. Goutman, Adam Quick, Stephen J. Kolb & Jennifer Roggenbuck
Pages: 216-221
Published online: 01 Apr 2019
AbstractforLack of consensus in ALS genetic testing practices and divergent views between ALS clinicians and patients | Full Text | References | PDF (137.1 KB) | EPUB
  • 362 Views
  • 18 CrossRef citations
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Research Article

Article
Identification and functional analysis of novel mutations in the SOD1 gene in Chinese patients with amyotrophic lateral sclerosis
Hui-Xia Lin, Qing-Qing Tao, Qiao Wei, Cong-Xin Chen, Yu-Chao Chen, Hong-Fu Li, Aaron D. Gitler & Zhi-Ying Wu
Pages: 222-228
Published online: 19 Mar 2019
AbstractforIdentification and functional analysis of novel mutations in the SOD1 gene in Chinese patients with amyotrophic lateral sclerosis | Full Text | References | PDF (1 MB) | EPUB | Supplemental
  • 351 Views
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Article
Telomere length is greater in ALS than in controls: a whole genome sequencing study
Ahmad Al Khleifat, Alfredo Iacoangeli, Aleksey Shatunov, Ton Fang, William Sproviero, Ashley R. Jones, Sarah Opie-Martin, Karen E. Morrison, Pamela J. Shaw, Christopher E. Shaw, John F. Powell, Richard Dobson, Steven J. Newhouse & Ammar Al-Chalabi
Pages: 229-234
Published online: 01 Apr 2019
AbstractforTelomere length is greater in ALS than in controls: a whole genome sequencing study | Full Text | References | PDF (677.8 KB) | EPUB
  • 2378 Views
  • 17 CrossRef citations
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Open Access

Original Articles

Article
Psychological distress and coping styles of caregivers of patients with amyotrophic lateral sclerosis: a longitudinal study
Jessica de Wit, Leonhard A. Bakker, Annerieke C. van Groenestijn, Joost F. Baardman, Leonard H. van den Berg, Johanna M.A. Visser-Meily & Carin D. SchrÖder
Pages: 235-241
Published online: 18 Jan 2019
AbstractforPsychological distress and coping styles of caregivers of patients with amyotrophic lateral sclerosis: a longitudinal study | Full Text | References | PDF (781.3 KB) | EPUB
  • 2891 Views
  • 9 CrossRef citations
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Open Access
Article
Clinical audit research and evaluation of motor neuron disease (CARE-MND): a national electronic platform for prospective, longitudinal monitoring of MND in Scotland
Danielle Leighton, Judith Newton, Shuna Colville, Andrew Bethell, Gillian Craig, Laura Cunningham, Moira Flett, Dianne Fraser, Janice Hatrick, Helen Lennox, Laura Marshall, Dympna McAleer, Alison McEleney, Kitty Millar, Ann Silver, Laura Stephenson, Susan Stewart, Dorothy Storey, Gill Stott, Carol Thornton, Carolyn Webber, Harry Gordon, Giulia Melchiorre, Laura Sherlock, Emily Beswick, David Buchanan, Sharon Abrahams, Anthony Bateman, Jenny Preston, Callum Duncan, Richard Davenport, George Gorrie, Ian Morrison, Robert Swingler, Siddharthan Chandran & Suvankar Pal
Pages: 242-250
Published online: 20 Mar 2019
AbstractforClinical audit research and evaluation of motor neuron disease (CARE-MND): a national electronic platform for prospective, longitudinal monitoring of MND in Scotland | Full Text | References | PDF (468.8 KB) | EPUB
  • 645 Views
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Article
Amyotrophic lateral sclerosis among patients with a Medicare Advantage prescription drug plan; prevalence, survival and patient characteristics
Rituparna Bhattacharya, Raymond A. Harvey, Keli Abraham, Jon Rosen & Paul Mehta
Pages: 251-259
Published online: 20 Mar 2019
AbstractforAmyotrophic lateral sclerosis among patients with a Medicare Advantage prescription drug plan; prevalence, survival and patient characteristics | Full Text | References | PDF (541 KB) | EPUB | Supplemental
  • 2859 Views
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Open Access

Research Article

Article
Early post-marketing experience with edaravone in an unselected group of patients with ALS
Alon Abraham, Beatrice Nefussy, Yaara Fainmesser, Yoram Ebrahimi, Arnon Karni & Vivian E. Drory
Pages: 260-263
Published online: 20 Feb 2019
AbstractforEarly post-marketing experience with edaravone in an unselected group of patients with ALS | Full Text | References | PDF (178.4 KB) | EPUB
  • 535 Views
  • 15 CrossRef citations
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Article
Predicting the future of ALS: the impact of demographic change and potential new treatments on the prevalence of ALS in the United Kingdom, 2020–2116
Alison Gowland, Sarah Opie-Martin, Kirsten M. Scott, Ashley R. Jones, Puja R. Mehta, Christine J. Batts, Cathy M. Ellis, P. Nigel Leigh, Christopher E. Shaw, Jemeen Sreedharan & Ammar Al-Chalabi
Pages: 264-274
Published online: 09 Apr 2019
AbstractforPredicting the future of ALS: the impact of demographic change and potential new treatments on the prevalence of ALS in the United Kingdom, 2020–2116 | Full Text | References | PDF (901.7 KB) | EPUB | Supplemental
  • 5425 Views
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Open Access

Report

Report
Typical bulbar ALS can be linked to GARS mutation
P. Corcia, C. Brulard, S. Beltran, S. Marouillat, S.E. Bakkouche, C.R. Andres, H. Blasco & P. Vourc’h
Pages: 275-277
Published online: 20 Jan 2019
AbstractforTypical bulbar ALS can be linked to GARS mutation | Full Text | References | PDF (523 KB) | EPUB
  • 265 Views
  • 6 CrossRef citations
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Report
FUS-ALS presenting with myoclonic jerks in a 17-year-old man
Katherine Claire Dodd, Rachael Power, John Ealing & Hisham Hamdalla
Pages: 278-280
Published online: 18 Mar 2019
AbstractforFUS-ALS presenting with myoclonic jerks in a 17-year-old man | Full Text | References | PDF (1.2 MB) | EPUB
  • 272 Views
  • 9 CrossRef citations
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Short Report

Report
Cerebellar tract alterations in PLS and ALS
Sicong Tu, Ricarda A. L. Menke, Kevin Talbot, Matthew C. Kiernan & Martin R. Turner
Pages: 281-284
Published online: 21 Jan 2019
AbstractforCerebellar tract alterations in PLS and ALS | Full Text | References | PDF (711.3 KB) | EPUB
  • 352 Views
  • 21 CrossRef citations
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Research Article

Article
Lunasin does not slow ALS progression: results of an open-label, single-center, hybrid-virtual 12-month trial
R.S. Bedlack, Paul Wicks, Timothy Vaughan, Alicia Opie, Rebecca Blum, Amanda Dios & Ghazaleh Sadri-Vakili
Pages: 285-293
Published online: 21 Jan 2019
AbstractforLunasin does not slow ALS progression: results of an open-label, single-center, hybrid-virtual 12-month trial | Full Text | References | PDF (702.2 KB) | EPUB
  • 9423 Views
  • 15 CrossRef citations
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Open Access
Article
ALSUntangled No. 47: RT001
Richard Bedlack
Pages: 294-297
Published online: 28 Jan 2019
First Page PreviewforALSUntangled No. 47: RT001 | Full Text | References | PDF (115.6 KB) | EPUB
  • 1851 Views
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Free Access

Letter to the Editor

Letter
Edaravone administration in pivotal clinical Study 19
Angela Genge & Benjamin Brooks
Pages: 298-299
Published online: 13 May 2019
CitationforEdaravone administration in pivotal clinical Study 19 | Full Text | References | PDF (114.9 KB) | EPUB
  • 208 Views
  • 1 CrossRef citations
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Author’s Reply

Letter
Author response to a Letter to the Editor entitled: Edaravone administration in pivotal clinical study 19 (Authors: Genge, Angela; Brooks, Benjamin)
John Turnbull
Pages: 300-302
Published online: 10 May 2019
First Page PreviewforAuthor response to a Letter to the Editor entitled: Edaravone administration in pivotal clinical study 19 (Authors: Genge, Angela; Brooks, Benjamin) | Full Text | References | PDF (106.7 KB) | EPUB
  • 278 Views
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