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Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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https://www.readbyqxmd.com/read/30001159/deconstructing-progression-of-amyotrophic-lateral-sclerosis-in-stages-a-markov-modeling-approach
#1
Nimish J Thakore, Brittany R Lapin, Tyler G Kinzy, Erik P Pioro
OBJECTIVES: Propose an empirical amyotrophic lateral sclerosis (ALS) staging approach called Fine'til 9 (FT9) based on how many of the patient's ALS functional rating scale (ALSFRS-R) subscores are 9 or less (of normal 12). Gain insights into progression of ALS by applying Markov models to ALS stages by multiple systems (King's, Milan-Torino system (MITOS) and FT9). METHODS: Patients from the Pooled Resource Open-Access ALS Clinical Trials (PRO-ACT) dataset were staged using ALSFRS-R responses...
July 12, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29862846/multicenter-validation-of-18-f-fdg-pet-and-support-vector-machine-discriminant-analysis-in-automatically-classifying-patients-with-amyotrophic-lateral-sclerosis-versus-controls
#2
Ludovic D'hulst, Donatienne Van Weehaeghe, Adriano Chiò, Andrea Calvo, Cristina Moglia, Antonio Canosa, Angelina Cistaro, Stefanie Ma Willekens, Joke De Vocht, Philip Van Damme, Marco Pagani, Koen Van Laere
OBJECTIVE: 18 F-Fluorodeoxyglucose (18 F-FDG) positron emission tomography (PET) single-center studies using support vector machine (SVM) approach to differentiate amyotrophic lateral sclerosis (ALS) from controls have shown high overall accuracy on an individual patient basis using local a priori defined classifiers. The aim of the study was to validate the SVM accuracy on a multicentric level. METHODS: A previously defined Belgian (BE) group of 175 ALS patients (61...
June 4, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29848125/hope-and-self-efficacy-are-associated-with-better-satisfaction-with-life-in-people-with-als
#3
Shir Galin, Irit Heruti, Noa Barak, Marc Gotkine
The psychological phenotype in amyotrophic lateral sclerosis (ALS) is less negative than in other neurodegenerative diseases, manifested by a lower prevalence of psychopathology, such as anxiety and major depression, and a higher perceived quality of life by patients, irrespective of physical impairment. We hypothesized that positive psychological factors such as hope, optimism, and self-efficacy in people with ALS (PALS) were key determinants of satisfaction with life (SWL), despite physical impairment, and were protective against psychopathology...
May 31, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29693424/alsuntangled-45-antiretrovirals
#4
(no author information available yet)
No abstract text is available yet for this article.
April 25, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29804470/cognitive-behavioral-longitudinal-assessment-in-als-the-italian-edinburgh-cognitive-and-behavioral-als-screen-ecas
#5
Barbara Poletti, Federica Solca, Laura Carelli, Andrea Faini, Fabiana Madotto, Annalisa Lafronza, Alessia Monti, Stefano Zago, Andrea Ciammola, Antonia Ratti, Nicola Ticozzi, Sharon Abrahams, Vincenzo Silani
OBJECTIVE: The study presents data on the longitudinal administration of the Italian Edinburgh Cognitive and Behavioral ALS Screen (ECAS). We investigated cognitive-behavioral performance in a group of ALS patients over time and the feasibility of repeating the ECAS longitudinally compared with standard neuropsychological tests. Finally, correlations between clinical/genetic and cognitive/behavioral data were considered. METHODS: One hundred and sixty-eight ALS patients were tested at baseline (T0 )...
August 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29707972/corrigendum
#6
(no author information available yet)
No abstract text is available yet for this article.
August 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29564924/identification-of-an-a4v-sod1-mutation-in-a-chinese-patient-with-amyotrophic-lateral-sclerosis-without-the-a4v-founder-effect-common-in-north-america
#7
Lu Tang, Yan Ma, Xiaolu Liu, Lu Chen, Dongsheng Fan
We identified a missense alanine to valine mutation at codon 4 (A4V) in the Cu/Zn superoxide dismutase (SOD1) gene in a 51-year-old male of Chinese origin with familial amyotrophic lateral sclerosis (ALS). The patient displayed a typical A4V-related phenotype that included rapid progression and predominant lower motor neuron involvement. This patient is the first such carrier reported outside Caucasian ALS patients, despite the fact that A4V mutations account for up to 50% of all SOD1 mutations in North America...
August 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29411640/screening-for-possible-oligogenic-pathogenesis-in-chinese-sporadic-als-patients
#8
Hang Zhang, Wanshi Cai, Siyu Chen, Jialong Liang, Zhanjun Wang, Yuting Ren, Wenxiu Liu, Xiaolan Zhang, Zhongsheng Sun, Xusheng Huang
We investigated all coding regions of 17 known amyotrophic lateral sclerosis (ALS)-related genes in 311 sporadic ALS patients who were of Chinese ancestry using next-generation sequencing technology. All nonsynonymous variants identified were confirmed by Sanger sequencing. 29 (9.32%) patients harbored at least one pathogenic or likely pathogenic variants. Nine (2.8%) patients harbored two or three variants which frequency <1% in population databases that may be related to oligogenic pathogenesis. A higher allele frequency was observed in East Asian than in European patients for the majority variants identified in this screening, which may indicate that genetic factors are responsible for the different clinical characteristics between Chinese and European ALS patients...
August 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29384411/mass-spectrometry-analysis-of-plasma-from-amyotrophic-lateral-sclerosis-and-control-subjects
#9
Zhouwei Xu, Aven Lee, Amanda Nouwens, Robert David Henderson, Pamela Ann McCombe
Mass spectrometry was used to study blood samples from patients with amyotrophic lateral sclerosis (ALS) and healthy controls. Addenbrooke's cognitive examination-III (ACE-III) was used to test for cognitive impairment (CI). Nano liquid chromatography and time of flight mass spectrometry (MS) were performed on samples from 42 ALS patients and 18 healthy controls. SWATH™ proteomic analysis was utilized to look for differences between groups. Western blot analysis was used to study levels of 4 proteins, selected as being of possible interest in ALS, in the MS discovery cohort and a second validation group of 10 ALS patients and 10 healthy controls...
August 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29260601/an-association-study-between-scfd1-rs10139154-variant-and-amyotrophic-lateral-sclerosis-in-a-chinese-cohort
#10
Yongping Chen, Qingqing Zhou, Xiaojing Gu, Qianqian Wei, Bei Cao, Hui Liu, Yanbing Hou, Huifang Shang
BACKGROUND: A recent genome-wide association study (GWAS) demonstrated that the Sec1 family domain containing 1 (SCFD1) gene is associated with amyotrophic lateral sclerosis (ALS). The objective of our study was to investigate the association between the single nucleotide polymorphism (SNP) rs10139154 in the SCFD1 gene and ALS in a Chinese cohort. METHODS: A cohort of 1074 sporadic ALS (SALS) patients from the Department of Neurology at the West China Hospital of Sichuan University were genotyped for rs10139154 using a polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) analysis...
August 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29488394/erratum
#11
(no author information available yet)
No abstract text is available yet for this article.
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29402141/vitality-als-a-phase-iii-trial-of-tirasemtiv-a-selective-fast-skeletal-muscle-troponin-activator-as-a-potential-treatment-for-patients-with-amyotrophic-lateral-sclerosis-study-design-and-baseline-characteristics
#12
Jinsy A Andrews, Merit E Cudkowicz, Orla Hardiman, Lisa Meng, Amy Bian, Jacqueline Lee, Andrew A Wolff, Fady I Malik, Jeremy M Shefner
OBJECTIVE: To assess the efficacy of tirasemtiv, a fast skeletal muscle troponin activator, vs. placebo on respiratory function and other functional measures in patients with amyotrophic lateral sclerosis (ALS). This study was designed to confirm and extend results from a large phase IIb trial and maximize tolerability with a slower dose escalation. METHODS: VITALITY-ALS (NCT02496767) was a multinational, double-blind, randomized, placebo-controlled, parallel-group study in ALS patients...
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29383960/spatial-analyses-of-als-incidence-in-denmark-over-three-decades
#13
Verónica M Vieira, Johnni Hansen, Ole Gredal, Marc G Weisskopf
OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of the motor neuron with very few known risk factors. We conducted a spatial epidemiologic analysis of ALS incidence in Denmark to assess the contribution of sociodemographic determinants to geographic variation. METHODS: We analyzed 4249 ALS cases (1982-2013), each with 100 controls matched on sex and birth year. Odds ratio and 95% confidence bands at birth and diagnosis/index locations were calculated using generalized additive models...
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29382218/motor-neuron-disease-mortality-rates-in-new-zealand-1992-2013
#14
Maize C Cao, Andrew Chancellor, Alison Charleston, Mike Dragunow, Emma L Scotter
BACKGROUND: We determined the mortality rates of motor neuron disease (MND) in New Zealand over 22 years from 1992 to 2013. Previous studies have found an unusually high and/or increasing incidence of MND in certain regions of New Zealand; however, no studies have examined MND rates nationwide to corroborate this. METHODS: Death certificate data coded G12.2 by International Classification of Diseases (ICD)-10 coding, or 335.2 by ICD-9 coding were obtained. These codes specify amyotrophic lateral sclerosis, progressive bulbar palsy, or other motor neuron diseases as the underlying cause of death...
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29334251/discussing-edaravone-with-the-als-patient-an-ethical-framework-from-a-u-s-perspective
#15
Crystal Jing Jing Yeo, Zachary Simmons
The recent approval of edaravone by the United States Food and Drug Administration has generated a mix of hope tempered by reality. The costs of the drug, both monetarily and with regard to intensity of treatment, are high. The benefits, while modest, will be viewed through a very different lens by individuals depending on their goals of care. By virtue of our training and experience, physicians are ideally suited to understand and explain new treatments to our patients. As healthcare providers with a fiduciary responsibility to our patients, we must make sure they are fully informed about both the costs and benefits of non-curative therapies such as edaravone, and be prepared to discuss these in the context of their goals of care and potential impact on quality of life...
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29308669/an-open-label-study-of-a-novel-immunosuppression-intervention-for-the-treatment-of-amyotrophic-lateral-sclerosis
#16
Christina N Fournier, David Schoenfeld, James D Berry, Merit E Cudkowicz, James Chan, Colin Quinn, Robert H Brown, Johnny S Salameh, Malu G Tansey, David R Beers, Stanley H Appel, Jonathan D Glass
Neuroinflammation is increasingly tied to disease progression in amyotrophic lateral sclerosis (ALS). Participants in the first-in-human trial of intra-spinal allogeneic stem cell therapy for ALS received immunosuppression, and one participant saw dramatic improvement across multiple outcome measures. The primary objective of this study (NCT01884571) was to assess the rate of clinical response to the same immunosuppressive regimen using basiliximab, tacrolimus, mycophenolate, and prednisone in people with ALS...
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29277115/pre-diagnostic-plasma-urate-and-the-risk-of-amyotrophic-lateral-sclerosis
#17
Éilis J O'Reilly, Kjetil Bjornevik, Michael A Schwarzschild, Marjorie L McCullough, Laurence N Kolonel, Loic Le Marchand, Joann E Manson, Alberto Ascherio
OBJECTIVE: To prospectively examine for the first time the association between plasma urate levels measured in healthy participants and future amyotrophic lateral sclerosis (ALS) risk. METHODS: A pooled case-control study nested in five US prospective cohorts comprising 319,617 participants who provided blood, of which 275 had ALS during follow-up. Pre-diagnostic plasma urate was determined for all participants using a clinical colorimetric enzyme assay. Gender-specific multivariable-adjusted rate ratios (RR) of ALS incidence or death estimated by conditional logistic regression and pooled using inverse-variance weighting...
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29271248/assessing-amyotrophic-lateral-sclerosis-prevalence-in-norway-from-2009-to-2015-from-compulsory-nationwide-health-registers
#18
Ola Nakken, Jonas Christoffer Lindstrøm, Ole-Bjørn Tysnes, Trygve Holmøy
OBJECTIVE: In Norway, diagnoses from specialist health care visits, drug prescriptions, and causes of deaths are registered in compulsory health registers. We aimed to determine amyotrophic lateral sclerosis (ALS) prevalence from 2009 to 2015 by combining these registers. METHODS: We validated the Norwegian Patient Registry (NPR) through hospital files, and linked it with the Norwegian Cause of Death Registry and the Norwegian Prescription Database. Poisson regression models were fitted for estimating gender ratios, time trends and possible interactions...
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29268633/amyotrophic-lateral-sclerosis-and-food-intake
#19
Elisabetta Pupillo, Elisa Bianchi, Adriano Chiò, Federico Casale, Chiara Zecca, Rosanna Tortelli, Ettore Beghi
OBJECTIVE: To verify if specific foods and nutrients could be risk factors or protective factors for amyotrophic lateral sclerosis (ALS). METHODS: Patients with newly diagnosed ALS from three Italian administrative regions were included. For each patient, a healthy control, matched for age (±5 years), sex and administrative region of residence, was selected by a general practitioner. Cases and controls were interviewed by a trained investigator who filled a validated and reproducible food-frequency questionnaire...
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29260911/a-retrospective-investigation-of-the-relationship-between-baseline-covariates-and-rate-of-alsfrs-r-decline-in-als-clinical-trials
#20
Iyas Daghlas, Teresa E Lever, Emily Leary
The revised ALS functional rating scale (ALSFRS-R) is a longitudinal measure of global function commonly used to assess progression of amyotrophic lateral sclerosis (ALS), and as an endpoint in ALS clinical trials. Understanding how baseline covariates affect the rate of functional decline in ALS offers valuable information to clinical trialists. We used a mixed modeling approach in a retrospective study of the pooled resource open-Access ALS clinical trials database to elucidate the associations between baseline covariates and the rate of ALSFRS-R decline over time...
May 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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