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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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https://www.readbyqxmd.com/read/28618842/alsuntangled-40-ayahuasca
#1
(no author information available yet)
No abstract text is available yet for this article.
June 15, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28616937/influence-of-arterial-hypertension-type-2-diabetes-and-cardiovascular-risk-factors-on-als-outcome-a-population-based-study
#2
Cristina Moglia, Andrea Calvo, Antonio Canosa, Davide Bertuzzo, Paolo Cugnasco, Luca Solero, Maurizio Grassano, Enrica Bersano, Stefania Cammarosano, Umberto Manera, Fabrizio Pisano, Letizia Mazzini, Laura A Dalla Vecchia, Gabriele Mora, Adriano Chiò
OBJECTIVE: To assess the prognostic influence of pre-morbid type 2 diabetes mellitus, arterial hypertension and cardiovascular (CV) risk profile on ALS phenotype and outcome in a population-based cohort of Italian patients. METHODS: A total of 650 ALS patients from the Piemonte/Valle d'Aosta Register for ALS, incident in the 2007-2011 period, were recruited. Information about premorbid presence of type 2 diabetes mellitus, arterial hypertension was collected at the time of diagnosis...
June 15, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28616933/split-hand-index-and-ulnar-to-median-ratio-in-hirayama-disease-and-amyotrophic-lateral-sclerosis
#3
Jayantee Kalita, Surendra Kumar, Usha K Misra, Zafar Neyaz
OBJECTIVE: We report the relative usefulness of split hand index (SHI) and ulnar to median (UM) ratio in Hirayama disease (HD) and amyotrophic lateral sclerosis (ALS). METHODS: The patients with HD, ALS and matched controls were subjected to evaluation of UM ratio and SHI. Compound muscle action potentials (CMAPs) were recorded from abductor digit minimi (ADM) and first dorsal interosseous (FDI) by stimulating ulnar nerve at the wrist, and abductor pollicis brevis (APB) by stimulating median nerve at the wrist...
June 15, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28597697/effectiveness-of-assisted-and-unassisted-cough-capacity-in-amyotrophic-lateral-sclerosis-patients
#4
Jesús Sancho, Emilio Servera, Pilar Bañuls, Julio Marín
Decreased cough capacity during a respiratory infection is one of the main causes of acute respiratory failure and hospitalisation in amyotrophic lateral sclerosis (ALS). OBJECTIVE: To determine whether a respiratory measurement could identify the effectiveness of cough capacity in ALS during a respiratory infection. METHODS: This was a prospective study of all ALS patients who were treated at a respiratory care unit due to a respiratory infection from 2012 to 2016...
June 9, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28593798/nocebo-in-motor-neuron-disease-systematic-review-and-meta-analysis-of-placebo-controlled-clinical-trials
#5
Faiza Shafiq, Dimos-Dimitrios Mitsikostas, Panagiotis Zis
INTRODUCTION: Nocebo is very prevalent among neurological disorders, resulting in low adherence and treatment outcome. We sought to examine the AEs following placebo administration in placebo-controlled randomised clinical trials (RCTs) for motor neuron disease (MND). METHODS: After a systematic literature search for RCTs for MND pharmacotherapy treatments, we assessed the number of discontinuations because of placebo intolerance. RESULTS: Data were extracted from 12 RCTs fulfilling our search criteria...
June 8, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28585888/predictive-genetic-testing-for-amyotrophic-lateral-sclerosis-and-frontotemporal-dementia-genetic-counselling-considerations
#6
Ashley Crook, Kelly Williams, Lorel Adams, Ian Blair, Dominic B Rowe
Once a gene mutation that is causal of amyotrophic lateral sclerosis (ALS) and/or frontotemporal dementia (FTD) is identified in a family, relatives may decide to undergo predictive genetic testing to determine whether they are at risk of developing disease. Recent advances in gene discovery have led to a pressing need to better understand the implications of predictive genetic testing. Here we review the uptake of genetic counselling, predictive and reproductive testing, and the factors that impact the decision to undergo testing, for consideration in clinical practice...
June 6, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28585472/home-care-organization-impacts-patient-management-and-survival-in-als
#7
Sylvie Lavernhe, Jean-Christophe Antoine, Isabelle Court-Fortune, Nathalie Dimier, Frédéric Costes, Arnaud Lacour, Jean-Philippe Camdessanché
OBJECTIVE: Progression of amyotrophic lateral sclerosis (ALS) depends on several factors linked to the disease. However, both the patient's living place and care organization role need to be evaluated. METHODS: We analysed the effect on survival of factors linked to ALS or the socio-geographical context in a prospective cohort of 203 patients followed between 2003 and 2011. RESULTS: Patients were 97 females and 106 males with a mean age of 65...
June 6, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28569083/magnetic-fields-exposure-from-high-voltage-power-lines-and-risk-of-amyotrophic-lateral-sclerosis-in-two-italian-populations
#8
Marco Vinceti, Carlotta Malagoli, Sara Fabbi, Leeka Kheifets, Federica Violi, Maurizio Poli, Salvatore Caldara, Daniela Sesti, Silvia Violanti, Paolo Zanichelli, Barbara Notari, Roberto Fava, Alessia Arena, Roberta Calzolari, Tommaso Filippini, Laura Iacuzio, Elisa Arcolin, Jessica Mandrioli, Nicola Fini, Anna Odone, Carlo Signorelli, Francesco Patti, Mario Zappia, Vladimiro Pietrini, Paola Oleari, Sergio Teggi, Grazia Ghermandi, Angela Dimartino, Caterina Ledda, Cristina Mauceri, Salvatore Sciacca, Maria Fiore, Margherita Ferrante
The aetiology of amyotrophic lateral sclerosis (ALS), a rare and extremely severe neurodegenerative disease, has been associated with magnetic fields exposure. However, evidence for such a relation in the general population is weak, although the previous null results might also be due to exposure misclassification, or a relationship might exist only for selected subgroups. To test such a hypothesis we carried out a population-based case-control study in two Northern and Southern Italy regions, including 703 ALS cases newly diagnosed from 1998 to 2011 and 2737 controls randomly selected from the residents in the study provinces...
June 1, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28562080/neuroimaging-patterns-along-the-als-ftd-spectrum-a-multiparametric-imaging-study
#9
Taha Omer, Eoin Finegan, Siobhan Hutchinson, Mark Doherty, Alice Vajda, Russell L McLaughlin, Niall Pender, Orla Hardiman, Peter Bede
Frontotemporal dementia is associated with considerable clinical, genetic and pathological heterogeneity. The objective of this study is to characterise the imaging signatures of the main FTD phenotypes along the ALS-FTD spectrum. A total of 100 participants underwent comprehensive multimodal neuroimaging, genetic testing and neuropsychological evaluation. Seven patients with behavioural variant FTD (bvFTD), 11 patients with non-fluent-variant primary progressive aphasia (nfvPPA), two patients with sematic-variant primary progressive aphasia(svPPA), 10 patients with amyotrophic lateral sclerosis and FTD carrying the C9orf72 hexanucleotide repeat (C9 + ALS-FTD), 10 patients with ALS-FTD without hexanucleotide repeats (C9-ALS-FTD), 20 ALS patients without behavioural or cognitive deficits (ALSnci) and 40 healthy controls (HC) were included in a prospective quantitative neuroimaging study...
May 31, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28562075/c9orf72-mutations-do-not-influence-the-tau-signature-of-amyotrophic-lateral-sclerosis-with-cognitive-impairment-alsci
#10
Kathryn Volkening, Wendy L Strong, Shauntel Seaton, Wencheng Yang, Michael J Strong
OBJECTIVE: C9orf72 mutations are associated with amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD) and ALS-FTD. In addition to ALS-FTD, ALS patients may develop a spectrum of neuropsychological and neuropsychiatric deficits including ALS with cognitive impairment (ALSci). Here we examine the extent to which C9orf72 mutations are associated with ALSci and whether this alters the tau molecular signature. METHODS: We identified 16 ALSci cases within a post-mortem archive of 94 fully genotyped ALS cases, eight of which harboured a C9orf72 mutation, in addition to three cognitively-intact ALS cases with a C9orf72 mutation...
May 31, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28513234/the-development-of-the-uk-national-institute-of-health-and-care-excellence-evidence-based-clinical-guidelines-on-motor-neurone-disease
#11
David Oliver, Aleksandar Radunovic, Alexander Allen, Christopher McDermott
The care of people with motor neuron disease/amyotrophic lateral sclerosis is often complex and involves a wide multidisciplinary team approach. The National Institute for Health and Care Excellence (NICE) in the UK has produced an evidence based guideline for the management of patients. This has made recommendations, based on clear evidence or consensus discussion. The evidence is often limited and areas for further research are suggested.
May 17, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28485644/impairment-of-triad-conditioned-facilitation-in-amyotrophic-lateral-sclerosis
#12
Stefan J Groiss, Hitoshi Mochizuki, Ritsuko Hanajima, Carlos Trenado, Setsu Nakatani-Enomoto, Koji Otani, Yoshikazu Ugawa
OBJECTIVES: The triad conditioned facilitation (TCF) technique has been shown to detect motor cortical intrinsic rhythms depending on the functioning of specific cortical layers by measuring motor evoked potential (MEP) enhancement after a triad of conditioning TMS pulses at a certain interval. However, the influence of cortical degeneration on TCF is still undetermined. We therefore studied TCF in patients with amyotrophic lateral sclerosis (ALS), a neurodegenerative disorder characterised by degeneration of the motor cortex...
May 9, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28457142/decision-making-for-tracheostomy-in-amyotrophic-lateral-sclerosis-als-a-retrospective-study
#13
Piero Ceriana, Sara Surbone, Daniele Segagni, Annia Schreiber, Annalisa Carlucci
BACKGROUND: ALS patients should discuss the issue of tracheostomy before the onset of terminal respiratory failure. While the process of shared decision-making is desirable, there are few data on the practical application of this real-life situation. AIM OF THE STUDY: To determine how a decision-making process is actually carried out, we analysed the episodes of acute respiratory failure preceding tracheostomy. METHODS: We studied the charts of a group of ALS patients after tracheostomy...
April 29, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28446030/anthropometric-measures-are-not-accurate-predictors-of-fat-mass-in-als
#14
Zara A Ioannides, Frederik J Steyn, Robert D Henderson, Pamela A Mccombe, Shyuan T Ngo
BACKGROUND: Anthropometric measurements including body mass index (BMI) and body adiposity index (BAI) are widely employed as indicators of fat mass (FM). Metabolic abnormalities in amyotrophic lateral sclerosis (ALS) impact disease progression, therefore assessment of FM informs care. The aim of this study was to determine whether BMI and BAI are accurate predictors of FM in ALS. Methodology and main findings: BMI, BAI and percentage FM (determined by air displacement plethysmography; FM-ADP) were measured in control (n = 35) and ALS (n = 44) participants...
April 27, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28440098/early-diagnosis-of-amyotrophic-lateral-sclerosis-mimic-syndromes-pros-and-cons-of-current-clinical-diagnostic-criteria
#15
Elena Cortés-Vicente, Jesús Pradas, Juan Marín-Lahoz, Noemi De Luna, Jordi Clarimón, Janina Turon-Sans, Ellen Gelpí, Jordi Díaz-Manera, Isabel Illa, Ricard Rojas-Garcia
OBJECTIVE: To describe the frequency and clinical characteristics of patients referred to a tertiary neuromuscular clinic as having amyotrophic lateral sclerosis (ALS) but who were re-diagnosed as having an ALS mimic syndrome, and to identify the reasons that led to the revision of the diagnosis. METHODS: We reviewed the final diagnosis of all patients prospectively registered in the Sant Pau-MND register from 1 January 2004 to 31 December 2015. A detailed clinical evaluation and a clinically-guided electrophysiological study were performed at first evaluation...
April 25, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28431489/apathy-in-amyotrophic-lateral-sclerosis-insights-from-dimensional-apathy-scale
#16
Gabriella Santangelo, Mattia Siciliano, Luigi Trojano, Cinzia Femiano, Maria Rosaria Monsurrò, Gioacchino Tedeschi, Francesca Trojsi
OBJECTIVES: Apathy is associated with cognitive decline and worse survival in amyotrophic lateral sclerosis (ALS); an accurate evaluation of this aspect is relevant in clinical settings. The aims of this study are to evaluate the prevalence of apathy in a large ALS sample, using published diagnostic criteria, and to explore the psychometric properties, the sensitivity and the specificity of the Dimensional Apathy Scale (DAS) as a screening tool for apathy. METHODS: One hundred and thirty-one patients underwent clinical interview based on diagnostic criteria for apathy, DAS, Apathy Evaluation Scale, and assessment of depression, global cognitive functioning, and non-verbal intelligence...
April 21, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28427284/delivering-tertiary-centre-specialty-care-to-als-patients-via-telemedicine-a-retrospective-cohort-analysis
#17
Stephen M Selkirk, Monique O Washington, Frances McClellan, Broderick Flynn, Jacinta M Seton, Richard Strozewski
OBJECTIVE: This study was undertaken to determine if ALS patients evaluated via telemedicine received the same quality of care as patients evaluated by traditional face-to-face encounters. METHODS: A retrospective cohort study design was used. Participants were patients diagnosed with ALS that received multidisciplinary care at the tertiary Cleveland VA ALS Centre between 1 March 2008- and 31 anuary 2015. Participants were not randomised, but chose telemedicine based on preference, disability level or distance from the clinic...
April 21, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28425762/the-evaluation-of-bulbar-dysfunction-in-amyotrophic-lateral-sclerosis-survey-of-clinical-practice-patterns-in-the-united-states
#18
Emily K Plowman, Lauren C Tabor, James Wymer, Gary Pattee
OBJECTIVE: Speech and swallowing impairments are highly prevalent in individuals with amyotrophic lateral sclerosis (ALS) and contribute to reduced quality of life, malnutrition, aspiration, pneumonia and death. Established practice parameters for bulbar dysfunction in ALS do not currently exist. The aim of this study was to identify current practice patterns for the evaluation of speech and swallowing function within participating Northeast ALS clinics in the United States. METHODS AND RESULTS: A 15-item survey was emailed to all registered NEALS centres...
April 20, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28420245/a-first-approach-to-a-neuropsychological-screening-tool-using-eye-tracking-for-bedside-cognitive-testing-based-on-the-edinburgh-cognitive-and-behavioural-als-screen
#19
Jürgen Keller, Amon Krimly, Lisa Bauer, Sarah Schulenburg, Sarah Böhm, Helena E A Aho-Özhan, Ingo Uttner, Martin Gorges, Jan Kassubek, Elmar H Pinkhardt, Sharon Abrahams, Albert C Ludolph, Dorothée Lulé
OBJECTIVE: Reliable assessment of cognitive functions is a challenging task in amyotrophic lateral sclerosis (ALS) patients unable to speak and write. We therefore present an eye-tracking based neuropsychological screening tool based on the Edinburgh Cognitive and Behavioural ALS Screen (ECAS), a standard screening tool for cognitive deficits in ALS. METHODS: In total, 46 ALS patients and 50 healthy controls matched for age, gender and education were tested with an oculomotor based and a standard paper-and-pencil version of the ECAS...
April 19, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28420241/percutaneous-endoscopic-gastrostomy-in-high-risk-als-insights-to-effort-to-drawing-a-risk-map
#20
Antonio M Esquinas, Giancarlo Garuti
No abstract text is available yet for this article.
April 19, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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