Hypermetabolism in ALS is associated with greater functional decline and shorter survival.
Frederik J. Steyn,Zara A. Ioannides,Ruben P A van Eijk,Susan Heggie,Kathryn A Thorpe,Amelia Ceslis,Saman Heshmat,Anjali K. Henders,Naomi R. Wray,Naomi R. Wray,Leonard H. van den Berg,Robert D. Henderson,Pamela A. McCombe,Shyuan T. Ngo +13 more
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TLDR
Hypermetabolic patients with ALS have a greater level of lower motor neuron involvement, faster rate of functional decline and shorter survival, and the metabolic index could be important for informing prognosis in ALS.Abstract:
Objective To determine the prevalence of hypermetabolism, relative to body composition, in amyotrophic lateral sclerosis (ALS) and its relationship with clinical features of disease and survival. Methods Fifty-eight patients with clinically definite or probable ALS as defined by El Escorial criteria, and 58 age and sex-matched control participants underwent assessment of energy expenditure. Our primary outcome was the prevalence of hypermetabolism in cases and controls. Longitudinal changes in clinical parameters between hypermetabolic and normometabolic patients with ALS were determined for up to 12 months following metabolic assessment. Survival was monitored over a 30-month period following metabolic assessment. Results Hypermetabolism was more prevalent in patients with ALS than controls (41% vs 12%, adjusted OR=5.4; p Conclusions and relevance Hypermetabolic patients with ALS have a greater level of lower motor neuron involvement, faster rate of functional decline and shorter survival. The metabolic index could be important for informing prognosis in ALS.read more
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Driven to decay: excitability and synaptic abnormalities in amyotrophic lateral sclerosis
TL;DR: A specific focus on a nexus point in ALS pathogenesis, namely, the synaptic and intrinsic hyperexcitability of CSMNs and MNs and alterations to their structure are comprehensively detailed and the association of extramotor dysfunction with neuronal structural/functional alterations will be discussed.
Journal ArticleDOI
Early weight loss in amyotrophic lateral sclerosis: outcome relevance and clinical correlates in a population-based cohort
Cristina Moglia,Andrea Calvo,Maurizio Grassano,Antonio Canosa,Umberto Manera,Fabrizio D'Ovidio,Alessandro Bombaci,Enrica Bersano,Letizia Mazzini,Gabriele Mora,Adriano Chiò +10 more
TL;DR: The rate of weight loss from onset to diagnosis was found to be a strong and independent prognostic factor in ALS and it is recommended that in clinical trials patients should be stratified according to the presence of dysphagia at the time of enrolment and not by site of onset of symptoms.
Journal ArticleDOI
Causal association of type 2 diabetes with amyotrophic lateral sclerosis: new evidence from Mendelian randomization using GWAS summary statistics
TL;DR: New evidence is provided supporting the causal neuroprotective role of T2D on ALS in the European population and empirically suggestive evidence of increasing risk of T1D onALS in the East Asian population.
Journal ArticleDOI
A Systematic Review of Suggested Molecular Strata, Biomarkers and Their Tissue Sources in ALS
Udaya Geetha Vijayakumar,Vanessa Milla,Mei Yu Cynthia Stafford,Anthony J. Bjourson,William Duddy,Stephanie Duguez +5 more
TL;DR: The present review group the molecular pathways that have been implicated in monogenic forms of ALS, to enable the description of patient strata corresponding to each pathway grouping, and suggests 14 strata, each potentially targetable by different pharmacological strategies.
Journal ArticleDOI
Skeletal-Muscle Metabolic Reprogramming in ALS-SOD1G93A Mice Predates Disease Onset and Is A Promising Therapeutic Target
Silvia Scaricamazza,Illari Salvatori,Giacomo Giacovazzo,Jean Philippe Loeffler,Jean Philippe Loeffler,Frédérique René,Frédérique René,Marco Rosina,Cyril Quessada,Cyril Quessada,Daisy Proietti,Constantin Heil,Simona Rossi,Simona Rossi,Stefania Battistini,Fabio Giannini,Nila Volpi,Frederik J. Steyn,Shyuan T. Ngo,Elisabetta Ferraro,Luca Madaro,Roberto Coccurello,Cristiana Valle,Alberto Ferri +23 more
TL;DR: It is shown that bioenergetic defects and muscle remodeling occur in skeletal muscle of the SOD1G93A mouse model of ALS mice prior to disease onset and before the activation of muscle denervation markers, respectively, and changes in muscle physiology were followed by an increase in energy expenditure unrelated to physical activity.
References
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Book
Multiple imputation for nonresponse in surveys
TL;DR: In this article, a survey of drinking behavior among men of retirement age was conducted and the results showed that the majority of the participants reported that they did not receive any benefits from the Social Security Administration.
Multiple Imputation For Nonresponse In Surveys
TL;DR: The multiple imputation for nonresponse in surveys is universally compatible with any devices to read and is available in the book collection an online access to it is set as public so you can download it instantly.
Journal ArticleDOI
El Escorial revisited : revised criteria for the diagnosis of amyotrophic lateral sclerosis
TL;DR: The criteria described below represent the result of a three-day workshop, convened at Airlie Conference Center, Warrenton, Virginia on 2–4 April, 1998 by the World Federation of Neurology Research Committee on Motor Neuron Diseases, and are placed on the WFN ALS website.
Journal ArticleDOI
The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function
Jesse M. Cedarbaum,Nancy Stambler,Errol Malta,Cynthia Fuller,Dana Hilt,Barbara Thurmond,Arline Nakanishi +6 more
TL;DR: A revised version of the ALSFRS, which incorporates additional assessments of dyspnea, orthopnea, and the need for ventilatory support is validated, indicating that the quality of function is a strong determinant of quality of life in ALS.
Book
Joint Models for Longitudinal and Time-to-Event Data: With Applications in R
TL;DR: This paper presents a meta-analysis of longitudinal and time-to-Event data patterns and discusses the role of the Parameterization on Predictions and Prospective Accuracy Measures for Longitudinal Markers in predicting survival and longitudinal outcomes.