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https://www.readbyqxmd.com/read/28227133/comparison-between-k-nearest-neighbor-approaches-for-conditional-entropy-estimation-application-to-the-assessment-of-the-cardiac-control-in-amyotrophic-lateral-sclerosis-patients
#1
Alberto Porta, Beatrice De Maria, Vlasta Bari, Andrea Marchi, Kalliopi Marinou, Riccardo Sideri, Gabriele Mora, Laura Dalla Vecchia, Alberto Porta, Beatrice De Maria, Vlasta Bari, Andrea Marchi, Kalliopi Marinou, Riccardo Sideri, Gabriele Mora, Laura Dalla Vecchia, Kalliopi Marinou, Gabriele Mora, Alberto Porta, Laura Dalla Vecchia, Riccardo Sideri, Beatrice De Maria, Vlasta Bari, Andrea Marchi
The study evaluates the k-nearest-neighbor (KNN) strategy for the assessment of complexity of the cardiac neural control from spontaneous fluctuations of heart period (HP). Two different procedures were assessed: i) the KNN estimation of the conditional entropy (CE) proposed by Porta et al; ii) the KNN estimation of mutual information proposed by Kozachenko-Leonenko, refined by Kraskov-Stögbauer-Grassberger and here adapted for the CE estimation. The two procedures were compared over HP variability recordings obtained at rest in supine position and during head-up tilt (HUT) in amyotrophic lateral sclerosis patients and healthy subjects...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28226830/on-the-development-of-a-low-cost-emg-switch-for-communication-using-minimal-muscle-contractions
#2
Begona Garcia-Conde, Christopher J James, Begona Garcia-Conde, Christopher J James, Begona Garcia-Conde, Christopher J James
In the final stages of muscle-wasting diseases like Amyotrophic Lateral Sclerosis and in extreme cases of disability the control of muscles is almost completely lost. However, in some cases there can be almost imperceptible residual control of some muscles. This paper presents the results of a study using an affordable, lightweight, portable EMG switch that has been designed in-house for communication in severely disabled or locked-in patients. It is possible to affect communication with the outside world by using these small residual muscle movements; in this case, more specifically muscles around the eyebrow...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28225381/information-seeking-behavior-and-information-needs-in-patients-with-amyotrophic-lateral-sclerosis-analyzing-an-online-patient-community
#3
Juyeon Oh, Jung A Kim
A few studies have examined the specific informational needs of the population with amyotrophic lateral sclerosis. The aims of this study were to describe the information-seeking behavior and information needs of patients with amyotrophic lateral sclerosis and their families in Korea by analyzing messages from an online patient community. A total of 1047 messages from the question and answer forum of the "Lou Gehrig's Disease Network" (http://cafe.daum.net/alsfree) from January 2010 to September 2015 were collected...
February 22, 2017: Computers, Informatics, Nursing: CIN
https://www.readbyqxmd.com/read/28222900/genetic-analysis-of-the-sod1-and-c9orf72-genes-in-hungarian-patients-with-amyotrophic-lateral-sclerosis
#4
Kornélia Tripolszki, Bernadett Csányi, Dóra Nagy, Antonia Ratti, Cinzia Tiloca, Vincenzo Silani, Éva Kereszty, Nóra Török, László Vécsei, József I Engelhardt, Péter Klivényi, Nikoletta Nagy, Márta Széll
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the death of motor neurons. To date, more than 20 genes have been implicated in ALS, and of these, the 2 most frequently mutated are the superoxide dismutase 1 (SOD1) gene and the chromosome 9 open reading frame 72 (C9ORF72) gene. In this study, we aimed to investigate the contribution of these 2 Mendelian genes to the development of the disease in Hungarian ALS patients (n = 66). Direct sequencing of the SOD1 gene revealed a novel (p...
January 29, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28222570/communication-vulnerable-in-patients-with-amyotrophic-lateral-sclerosis-a-systematic-review
#5
Lavoisier Leite Neto, Ana Carolina Constantini, Regina Yu Shon Chun
BACKGROUND: Individuals with Amyotrophic Lateral Sclerosis (ALS) exhibits speech disorders since the early stages that decrease the communication rate and interfere in social participation. OBJECTIVE: To conduct a literature review on communication vulnerable and Augmentative and Alternative Communication (AAC) in Amyotrophic Lateral Sclerosis. METHOD: Descriptors of the Health Sciences Descriptors (DeCS) were used: Amyotrophic Lateral Sclerosis, Health Vulnerability, Communication Barriers, Nonverbal Communication, and Communication Aids for Disabled...
February 10, 2017: NeuroRehabilitation
https://www.readbyqxmd.com/read/28222521/the-effects-of-lw-afc-on-the-hippocampus-transcriptome-in-senescence-accelerated-mouse-prone-8-strain-a-mouse-model-of-alzheimer-s-disease
#6
Jianhui Wang, Yang Liu, Xiaorui Cheng, Xiaorui Zhang, Feng Liu, Gang Liu, Shanyi Qiao, Ming Ni, Wenxia Zhou, Yongxiang Zhang, Fei Li
The senescence-accelerated mouse prone 8 (SAMP8) strain is considered a robust experimental model for developing preventative and therapeutic treatments for Alzheimer's disease (AD), a neurodegenerative disease which cannot be effectively prevented, halted, or cured. Our previous studies showed that LW-AFC, a new formula derived from the classical traditional Chinese medicinal prescription Liuwei Dihuang decoction, ameliorates cognitive deterioration in PrP-hAβPPswe/PS1ΔE9 transgenic mice and SAMP8 mice. This study aims to investigate the mechanism that mediates how LW-AFC improves cognitive deficit on the basis of the transcriptome...
February 10, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28220542/the-gut-microbiome-in-human-neurological-disease-a-review
#7
REVIEW
Helen Tremlett, Kylynda C Bauer, Silke Appel-Cresswell, Brett B Finlay, Emmanuelle Waubant
Almost half the cells and 1% of the unique genes found in our bodies are human, the rest are from microbes; predominantly bacteria, archaea, fungi, and viruses. These microorganisms collectively form the human microbiota, with most colonizing the gut. Recent technological advances, open access data-libraries, and application of high throughput sequencing have allowed these microbes to be identified and their contribution to neurological health examined. Emerging evidence links perturbations in the gut microbiota to neurological disease, including disease risk, activity, and progression...
February 21, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28220290/epidemiology-of-amyotrophic-lateral-sclerosis-in-southern-germany
#8
Angela Rosenbohm, Raphael S Peter, Siegfried Erhardt, Dorothée Lulé, Dietrich Rothenbacher, Albert C Ludolph, Gabriele Nagel
The objective of this study is to determine the current distribution of clinical phenotypes and to estimate future trends of ALS incidence in Western societies. We report on a clinical-epidemiological registry with a capture-recapture rate of >80% and population-based case-control study in ALS patients in South Western Germany. 1163 incidents of ALS were registered. Clinical and neuropsychological data were prospectively collected from 699 cases. The mean age at onset was 66.6 (SD = 11.6) years in prospective cases (N = 699)...
February 20, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28215142/small-molecule-modulation-of-hdac6-activity-the-propitious-therapeutic-strategy-to-vanquish-neurodegenerative-disorders
#9
Shabir Ahmad Ganai
Histone deacetylases (HDACs) are epigenetic enzymes creating the transcriptionally inactive state of chromatin by erasing acetyl moiety from histone and non-histone substrates. HDAC6 modulates several biological pathways in dividing cells as well as in post-mitotic neurons, and has been implicated in the pathophysiology of neurodegeneration. The distinct cellular functions and survival in these cells are reliant on HDAC6-mediated processes including intracellular trafficking, chaperone-mediated stress responses, anti-oxidation and protein degradation...
8, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28214109/a-novel-mutation-in-trem2-gene-causing-nasu-hakola-disease-and-review-of-the-literature
#10
Efthimios Dardiotis, Vasileios Siokas, Eva Pantazi, Maria Dardioti, Dimitrios Rikos, Georgia Xiromerisiou, Aikaterini Markou, Dimitra Papadimitriou, Matthaios Speletas, Georgios M Hadjigeorgiou
Nasu-hakola disease (NHD) is a rare disease characterized by bone cysts and fractures, frontal lobe syndrome, and progressive presenile dementia. NHD may be the prototype of primary microglial disorders of the CNS or, as they have been coined, "microgliopathies". Mutations in TREM2 and TYROBP genes are known to cause NHD. Interestingly, recent evidence-associated rare genetic variants of TREM2 gene with increased risk of Alzheimer's disease, frontotemporal dementia, amyotrophic lateral sclerosis, and Parkinson's disease...
January 20, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28213588/a-differential-autophagy-dependent-response-to-dna-double-strand-brakes-in-bone-marrow-mesenchymal-stem-cells-from-sporadic-als-patients
#11
Shane Wald-Altman, Edward Pichinuk, Or Kakhlon, Miguel Weil
Amyotrophic Lateral Sclerosis (ALS) is an incurable motor neurodegenerative disease caused by a diversity of genetic and environmental factors leading to neuromuscular degeneration and pathophysiological implications in non-neural systems. Our previous work showed abnormal transcriptional expression levels of biomarker genes in non-neuronal cell samples from ALS patients. The same genes proved to be differentially expressed in brain, spinal cord and muscle of the SOD1(G93A) ALS mouse model. These observations support the pathophysiological relevance of the ALS biomarkers discovered in human mesenchymal stem cells (hMSC) isolated from bone marrow samples of ALS patients (ALS-hMSC)...
February 16, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28210983/multi-view-ensemble-classification-of-brain-connectivity-images-for-neurodegeneration-type-discrimination
#12
Michele Fratello, Giuseppina Caiazzo, Francesca Trojsi, Antonio Russo, Gioacchino Tedeschi, Roberto Tagliaferri, Fabrizio Esposito
Brain connectivity analyses using voxels as features are not robust enough for single-patient classification because of the inter-subject anatomical and functional variability. To construct more robust features, voxels can be aggregated into clusters that are maximally coherent across subjects. Moreover, combining multi-modal neuroimaging and multi-view data integration techniques allows generating multiple independent connectivity features for the same patient. Structural and functional connectivity features were extracted from multi-modal MRI images with a clustering technique, and used for the multi-view classification of different phenotypes of neurodegeneration by an ensemble learning method (random forest)...
February 16, 2017: Neuroinformatics
https://www.readbyqxmd.com/read/28210978/future-directions-in-imaging-neurodegeneration
#13
REVIEW
Joseph C Masdeu
Neuroimaging comprises a powerful set of instruments to diagnose various neurodegenerative disorders, clarifies their neurobiology, and monitors their treatment. Magnetic resonance imaging depicts volume changes, as well as abnormalities in functional and structural connectivity. Positron emission tomography (PET) allows for the quantification of regional cerebral metabolism, characteristically altered in Alzheimer's disease, amyotrophic lateral sclerosis, diffuse Lewy-body disease, and the frontotemporal dementias...
January 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28209726/therapeutic-targeting-of-the-pathological-triad-of-extrasynaptic-nmda-receptor-signaling-in-neurodegenerations
#14
REVIEW
Hilmar Bading
Activation of extrasynaptic N-methyl-d-aspartate (NMDA) receptors causes neurodegeneration and cell death. The disease mechanism involves a pathological triad consisting of mitochondrial dysfunction, loss of integrity of neuronal structures and connectivity, and disruption of excitation-transcription coupling caused by CREB (cyclic adenosine monophosphate-responsive element-binding protein) shut-off and nuclear accumulation of class IIa histone deacetylases. Interdependency within the triad fuels an accelerating disease progression that culminates in failure of mitochondrial energy production and cell loss...
February 16, 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28208729/altered-intracellular-milieu-of-adar2-deficient-motor-neurons-in-amyotrophic-lateral-sclerosis
#15
REVIEW
Takenari Yamashita, Megumi Akamatsu, Shin Kwak
Transactive response DNA-binding protein (TDP-43) pathology, and failure of A-to-I conversion (RNA editing) at the glutamine/arginine (Q/R) site of α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor subunit GluA2, are etiology-linked molecular abnormalities that concomitantly occur in the motor neurons of most patients with amyotrophic lateral sclerosis (ALS). Adenosine deaminase acting on RNA 2 (ADAR2) specifically catalyzes GluA2 Q/R site-RNA editing. Furthermore, conditional ADAR2 knockout mice (AR2) exhibit a progressive ALS phenotype with TDP-43 pathology in the motor neurons, which is the most reliable pathological marker of ALS...
February 8, 2017: Genes
https://www.readbyqxmd.com/read/28208059/designer-protein-disaggregases-to-counter-neurodegenerative-disease
#16
REVIEW
James Shorter
Protein misfolding and aggregation unify several devastating neurodegenerative disorders, including Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis. There are no effective therapeutics for these disorders and none that target the reversal of the aberrant protein misfolding and aggregation that cause disease. Here, I showcase important advances to define, engineer, and apply protein disaggregases to mitigate deleterious protein misfolding and counter neurodegeneration. I focus on two exogenous protein disaggregases, Hsp104 from yeast and gene 3 protein from bacteriophages, as well as endogenous human protein disaggregases, including: (a) Hsp110, Hsp70, Hsp40, and small heat-shock proteins; (b) HtrA1; and (c) NMNAT2 and Hsp90...
February 13, 2017: Current Opinion in Genetics & Development
https://www.readbyqxmd.com/read/28205575/cu-ii-atsm-improves-the-neurological-phenotype-and-survival-of-sod1-g93a-mice-and-selectively-increases-enzymatically-active-sod1-in-the-spinal-cord
#17
James B Hilton, Stephen W Mercer, Nastasia K H Lim, Noel G Faux, Gojko Buncic, Joseph S Beckman, Blaine R Roberts, Paul S Donnelly, Anthony R White, Peter J Crouch
Ubiquitous expression of mutant Cu/Zn-superoxide dismutase (SOD1) selectively affects motor neurons in the central nervous system (CNS), causing the adult-onset degenerative disease amyotrophic lateral sclerosis (ALS). The CNS-specific impact of ubiquitous mutant SOD1 expression is recapitulated in transgenic mouse models of the disease. Here we present outcomes for the metallo-complex Cu(II)(atsm) tested for therapeutic efficacy in mice expressing SOD1(G93A) on a mixed genetic background. Oral administration of Cu(II)(atsm) delayed the onset of neurological symptoms, improved locomotive capacity and extended overall survival...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28203530/longitudinal-evaluation-of-cerebral-and-spinal-cord-damage-in-amyotrophic-lateral-sclerosis
#18
Milena de Albuquerque, Lucas Melo T Branco, Thiago Junqueira R Rezende, Helen Maia Tavares de Andrade, Anamarli Nucci, Marcondes Cavalcante França
OBJECTIVE: To evaluate MRI-based parameters as biomarkers of Amyotrophic Lateral Sclerosis (ALS) progression. METHODS: Twenty-seven patients and 27 controls performed two clinical and MRI acquisitions 8 months apart. ALSFRS-R scale was used to quantify disease severity at both time points. Multimodal analyses of MRI included cortical thickness measurements (FreeSurfer software), analysis of white matter integrity using diffusion-tensor imaging (tract-based spatial statistics-TBSS) and measurement of cervical spinal cord cross-sectional area (SpineSeg software)...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28202372/gut-microbiota-implications-in-parkinson-s-disease
#19
REVIEW
Arun Parashar, Malairaman Udayabanu
Gut microbiota (GM) can influence various neurological outcomes, like cognition, learning, and memory. Commensal GM modulates brain development and behavior and has been implicated in several neurological disorders like Alzheimer's disease, multiple sclerosis, amyotrophic lateral sclerosis, anxiety, stress and much more. A recent study has shown that Parkinson's disease patients suffer from GM dysbiosis, but whether it is a cause or an effect is yet to be understood. In this review, we try to connect the dots between GM and PD pathology using direct and indirect evidence...
February 7, 2017: Parkinsonism & related Disorders
https://www.readbyqxmd.com/read/28201634/occupations-and-amyotrophic-lateral-sclerosis-are-jobs-exposed-to-the-general-public-at-higher-risk
#20
F D'Ovidio, A d'Errico, A Calvo, G Costa, A Chiò
No abstract text is available yet for this article.
February 14, 2017: European Journal of Public Health
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