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https://www.readbyqxmd.com/read/27911333/the-%C3%A2-strand-ryhming-test-is-not-a-feasible-measure-in-ambulatory-patients-with%C3%A2-amyotrophic-lateral-sclerosis
#1
Annerieke C van Groenestijn, Olaf Verschuren, Carin D Schröder, Leonard H van den Berg, Johanna M A Visser-Meily
BACKGROUND: Ambulatory patients with Amyotrophic Lateral Sclerosis (ALS) show a decreased aerobic capacity which may hamper the ability to perform activities of daily living. A standardized measure, however, for assessing aerobic capacity in patients with ALS during the disease course, is lacking. OBJECTIVE: To examine the feasibility of the Åstrand-Ryhming (ÅR) test protocol longitudinally in ambulatory patients with amyotrophic lateral sclerosis (ALS). METHODS: Seven ambulatory male patients with spinal ALS onset were assessed at baseline and at 4, 7 and 10 months' follow-up...
November 29, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27910865/auditory-evoked-potential-could-reflect-emotional-sensitivity-and-impulsivity
#2
Ji Sun Kim, Sungkean Kim, Wookyoung Jung, Chang-Hwan Im, Seung-Hwan Lee
Emotional sensitivity and impulsivity could cause interpersonal conflicts and neuropsychiatric problems. Serotonin is correlated with behavioral inhibition and impulsivity. This study evaluated whether the loudness dependence of auditory evoked potential (LDAEP), a potential biological marker of central serotonergic activity, could reflect emotional sensitivity and impulsivity. A total of 157 healthy individuals were recruited, who performed LDAEP and Go/Nogo paradigms during electroencephalogram measurement...
December 2, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27909398/an-amyloid-like-pathological-conformation-of-tdp-43-is-stabilized-by-hypercooperative-hydrogen-bonds
#3
Miguel Mompeán, Marco Baralle, Emanuele Buratti, Douglas V Laurents
TDP-43 is an essential RNA-binding protein forming aggregates in almost all cases of sporadic amyotrophic lateral sclerosis (ALS) and many cases of frontotemporal lobar dementia (FTLD) and other neurodegenerative diseases. TDP-43 consists of a folded N-terminal domain with a singular structure, two RRM RNA-binding domains, and a long disordered C-terminal region which plays roles in functional RNA regulatory assemblies as well as pernicious aggregation. Evidence from pathological mutations and seeding experiments strongly suggest that TDP-43 aggregates are pathologically relevant through toxic gain-of-harmful-function and/or harmful loss-of-native-function mechanisms...
2016: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/27909307/motor-neuron-disease-brain-computer-interface-unlocks-the-mind-of-a-patient-with-als
#4
Heather Wood
No abstract text is available yet for this article.
December 2, 2016: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/27908811/decreased-serum-levels-of-high-mobility-group-box-1-hmgb-1-after-graft-replacement-or-stenting-of-abdominal-aortic-aneurysm
#5
Daiki Ousaka, Yasuhiro Fujii, Susumu Oozawa, Masahiro Nishibori, Yosuke Kuroko, Zenichi Masuda, Shunji Sano
OBJECTIVES: High-mobility group box 1 (HMGB-1) is a key substance mediating inflammation and development of atherosclerotic lesions (AL), including abdominal aortic aneurysms (AAA). Serum levels of HMGB-1 are increased in patients with AAA than in normal controls because the ALs in AAAs secrete HMGB-1. We therefore postulate that the serum HMGB-1 level should decrease after endovascular aortic repair (EVAR) or open aortic repair (OAR). However, there is no evidence of this in the literature...
November 28, 2016: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/27907080/development-of-an-automated-mri-based-diagnostic-protocol-for-amyotrophic-lateral-sclerosis-using-disease-specific-pathognomonic-features-a-quantitative-disease-state-classification-study
#6
Christina Schuster, Orla Hardiman, Peter Bede
BACKGROUND: Despite significant advances in quantitative neuroimaging, the diagnosis of ALS remains clinical and MRI-based biomarkers are not currently used to aid the diagnosis. The objective of this study is to develop a robust, disease-specific, multimodal classification protocol and validate its diagnostic accuracy in independent, early-stage and follow-up data sets. METHODS: 147 participants (81 ALS patients and 66 healthy controls) were divided into a training sample and a validation sample...
2016: PloS One
https://www.readbyqxmd.com/read/27906103/progressive-impairment-of-cav1-1-function-in-the-skeletal-muscle-of-mice-expressing-a-mutant-type-1-cu-zn-superoxide-dismutase-g93a-linked-to-amyotrophic-lateral-sclerosis
#7
Donald Beqollari, Christin F Romberg, Gabriella Dobrowolny, Martina Martini, Andrew A Voss, Antonio Musarò, Roger A Bannister
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder that is typically fatal within 3-5 years of diagnosis. While motoneuron death is the defining characteristic of ALS, the events that underlie its pathology are not restricted to the nervous system. In this regard, ALS muscle atrophies and weakens significantly before presentation of neurological symptoms. Since the skeletal muscle L-type Ca(2+) channel (CaV1.1) is a key regulator of both mass and force, we investigated whether CaV1...
June 23, 2016: Skeletal Muscle
https://www.readbyqxmd.com/read/27906081/vacht-overexpression-increases-acetylcholine-at-the-synaptic-cleft-and-accelerates-aging-of-neuromuscular-junctions
#8
Satoshi Sugita, Leland L Fleming, Caleb Wood, Sydney K Vaughan, Matheus P S M Gomes, Wallace Camargo, Ligia A Naves, Vania F Prado, Marco A M Prado, Cristina Guatimosim, Gregorio Valdez
BACKGROUND: Cholinergic dysfunction occurs during aging and in a variety of diseases, including amyotrophic lateral sclerosis (ALS). However, it remains unknown whether changes in cholinergic transmission contributes to age- and disease-related degeneration of the motor system. Here we investigated the effect of moderately increasing levels of synaptic acetylcholine (ACh) on the neuromuscular junction (NMJ), muscle fibers, and motor neurons during development and aging and in a mouse model for amyotrophic lateral sclerosis (ALS)...
October 5, 2016: Skeletal Muscle
https://www.readbyqxmd.com/read/27906075/nuclear-bodies-reorganize-during-myogenesis-in-vitro-and-are-differentially-disrupted-by-expression-of-fshd-associated-dux4
#9
Sachiko Homma, Mary Lou Beermann, Bryant Yu, Frederick M Boyce, Jeffrey Boone Miller
BACKGROUND: Nuclear bodies, such as nucleoli, PML bodies, and SC35 speckles, are dynamic sub-nuclear structures that regulate multiple genetic and epigenetic processes. Additional regulation is provided by RNA/DNA handling proteins, notably TDP-43 and FUS, which have been linked to ALS pathology. Previous work showed that mouse cell line myotubes have fewer but larger nucleoli than myoblasts, and we had found that nuclear aggregation of TDP-43 in human myotubes was induced by expression of DUX4-FL, a transcription factor that is aberrantly expressed and causes pathology in facioscapulohumeral dystrophy (FSHD)...
December 1, 2016: Skeletal Muscle
https://www.readbyqxmd.com/read/27905515/neonicotinoid-induced-impairment-of-odour-coding-in-the-honeybee
#10
Mara Andrione, Giorgio Vallortigara, Renzo Antolini, Albrecht Haase
Exposure to neonicotinoid pesticides is considered one of the possible causes of honeybee (Apis mellifera) population decline. At sublethal doses, these chemicals have been shown to negatively affect a number of behaviours, including performance of olfactory learning and memory, due to their interference with acetylcholine signalling in the mushroom bodies. Here we provide evidence that neonicotinoids can affect odour coding upstream of the mushroom bodies, in the first odour processing centres of the honeybee brain, i...
December 1, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27904119/an-autopsy-case-of-amyotrophic-lateral-sclerosis-with-diaphragm-pacing
#11
Hisashi Ito, Tetsumasa Kamei, Sanae Odake, Masayuki Nakano, Riki Okeda, Shunsaku Kohriki, Jun Kawachi, Raymond P Onders, Fumihito Yoshii
Respiratory insufficiency is a critical problem in amyotrophic lateral sclerosis (ALS) patients. We herein present the case of an autopsied patient with sporadic ALS who underwent diaphragm pacing (DP). The pathology showed several localized adhesions with a markedly atrophied diaphragm. A marked loss of motor neurons with Bunina bodies and phosphorylated TDP-43 positive inclusions was found in the spinal cord and primary motor cortex. Mild hyalinization and a few multinucleated giant cells were present around the electrode tracks in the diaphragm...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27902929/necroptosis-in-amyotrophic-lateral-sclerosis-and-other-neurological-disorders
#12
REVIEW
Jessica R Morrice, Cheryl Y Gregory-Evans, Christopher A Shaw
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the progressive degeneration of upper and lower motor neurons. Cell death in ALS and in general was previously believed to exist as a dichotomy between apoptosis and necrosis. Most research investigating cell death mechanisms in ALS was conducted before the discovery of programmed necrosis thus did not use selective cell death pathway-specific markers. Recently, a new form of programmed cell death, termed "necroptosis", has been characterized and has been recently implicated in ALS as a primary mechanism driving motor neuron cell death in different forms of ALS...
November 27, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27901255/assessment-and-nutrition-education-in-patients-with-amyotrophic-lateral-sclerosis
#13
Claudinéa S Almeida, Patricia Stanich, Cristina C S Salvioni, Solange Diccini
Neurological patients with amyotrophic lateral sclerosis (ALS)often deteriorate to a worsening nutritional status. The aim of this study was to compare the nutritional status and food intake after nutrition education in patients with ALS. Clinical, anthropometric and functional variables were analyzed. Fifty-three patients were monitored at an early stage of the disease. The average score on the functionality scale was 33 points. Initially only 3.8% were classified as low body weight. After three months, 50% showed significant variation in anthropometric measures related to muscle mass and body fat reserves without association with clinical variables...
November 2016: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/27900991/increased-expressions-of-plasma-galectin-3-in-patients-with-amyotrophic-lateral-sclerosis
#14
Jun Yan, Yun Xu, Li Zhang, Hui Zhao, Ling Jin, Wei-Guo Liu, Lei-Hua Weng, Zuo-Han Li, Ling Chen
BACKGROUND: High expressions of galectin-3 were identified recently in the end stage of amyotrophic lateral sclerosis (ALS) patients, which suggested that immune reactivity and inflammatory mechanisms might play an important role in the pathogenesis of ALS. The purpose of this study was to investigate plasma galectin-3 levels in different groups and stages of ALS patients and the association with related clinical characteristics. METHODS: A total of 51 patients with ALS and 60 normal controls (NCs) were recruited in this study...
2016: Chinese Medical Journal
https://www.readbyqxmd.com/read/27900375/aie-phenomena-of-a-cyanostilbene-derivative-as-a-probe-of-molecular-assembly-processes
#15
Fuyuki Ito, Jun-Ichi Fujimori, Narumi Oka, Michel Sliwa, Cyril Ruckebusch, Syoji Ito, Hiroshi Miyasaka
The initial processes of the crystallization of a solute molecule, 1-cyano-trans-1,2-bis-(4'-methylbiphenyl)-ethylene (CN-MBE) in binary solution (water and acetone), were investigated by means of fluorescence spectroscopy as well as scanning electron microscopy (SEM). With an increase in the volume fraction (Vw) of the poor solvent (water) in the solution, a drastic change in the fluorescence spectra and intensity of CN-MBE was observed. This change was attributed to aggregation induced emission (AIE). By analyzing the evolution of AIE by multivariate curve resolution-alternating least squares (MCR-ALS), it was revealed that four main species appeared in the solution depending on the Vw values...
November 30, 2016: Faraday Discussions
https://www.readbyqxmd.com/read/27899032/proteomic-profiling-of-the-spinal-cord-in-als-decreased-atp5d-levels-suggest-synaptic-dysfunction-in-als-pathogenesis
#16
Jooyeon Engelen-Lee, Anna M Blokhuis, Wim G M Spliet, R Jeroen Pasterkamp, Eleonora Aronica, Jeroen A A Demmers, Roel Broekhuizen, Giovanni Nardo, Niels Bovenschen, Leonard H Van Den Berg
BACKGROUND: We aimed to gain new insights into the pathogenesis of sporadic ALS (sALS) through a comprehensive proteomic analysis. METHODS: Protein profiles of the anterior and posterior horn in post-mortem spinal cord samples of 10 ALS patients and 10 controls were analysed using 2D-differential gel electrophoresis. The identified protein spots with statistically significant level changes and a spot ratio >2.0 were analysed by LC-MS/MS. RESULTS: In the posterior horn only 3 proteins were differentially expressed...
November 29, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27898262/characterization-of-genetic-loss-of-function-of-fus-in-zebrafish
#17
Svetlana Lebedeva, António M de Jesus Domingues, Falk Butter, René F Ketting
The RNA-binding protein FUS is implicated in transcription, alternative splicing of neuronal genes and DNA repair. Mutations in FUS have been linked to human neurodegenerative diseases such as ALS (amyotrophic lateral sclerosis). We genetically disrupted fus in zebrafish (Danio rerio) using the CRISPR-Cas9 system. The fus knockout animals are fertile and did not show any distinctive phenotype. Mutation of fus induces mild changes in gene expression on the transcriptome and proteome level in the adult brain...
November 29, 2016: RNA Biology
https://www.readbyqxmd.com/read/27897242/cortical-synaptic-and-dendritic-spine-abnormalities-in-a-presymptomatic-tdp-43-model-of-amyotrophic-lateral-sclerosis
#18
Matthew J Fogarty, Paul M Klenowski, John D Lee, Joy R Drieberg-Thompson, Selena E Bartlett, Shyuan T Ngo, Massimo A Hilliard, Mark C Bellingham, Peter G Noakes
Layer V pyramidal neurons (LVPNs) within the motor cortex integrate sensory cues and co-ordinate voluntary control of motor output. In amyotrophic lateral sclerosis (ALS) LVPNs and spinal motor neurons degenerate. The pathogenesis of neural degeneration is unknown in ALS; 10% of cases have a genetic cause, whereas 90% are sporadic, with most of the latter showing TDP-43 inclusions. Clinical and experimental evidence implicate excitotoxicity as a prime aetiological candidate. Using patch clamp and dye-filling techniques in brain slices, combined with high-resolution confocal microscopy, we report increased excitatory synaptic inputs and dendritic spine densities in early presymptomatic mice carrying a TDP-43(Q331K) mutation...
November 29, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27896976/missing-data-imputation-in-the-electronic-health-record-using-deeply-learned-autoencoders
#19
Brett K Beaulieu-Jones, Jason H Moore
Electronic health records (EHRs) have become a vital source of patient outcome data but the widespread prevalence of missing data presents a major challenge. Different causes of missing data in the EHR data may introduce unintentional bias. Here, we compare the effectiveness of popular multiple imputation strategies with a deeply learned autoencoder using the Pooled Resource Open-Access ALS Clinical Trials Database (PRO-ACT). To evaluate performance, we examined imputation accuracy for known values simulated to be either missing completely at random or missing not at random...
2016: Pacific Symposium on Biocomputing
https://www.readbyqxmd.com/read/27894372/affective-lability-mediates-the-association-between-childhood-trauma-and-suicide-attempts-mixed-episodes-and-co-morbid-anxiety-disorders-in-bipolar-disorders
#20
M Aas, C Henry, F Bellivier, M Lajnef, S Gard, J-P Kahn, T V Lagerberg, S R Aminoff, T Bjella, M Leboyer, O A Andreassen, I Melle, B Etain
BACKGROUND: Many studies have shown associations between a history of childhood trauma and more severe or complex clinical features of bipolar disorders (BD), including suicide attempts and earlier illness onset. However, the psychopathological mechanisms underlying these associations are still unknown. Here, we investigated whether affective lability mediates the relationship between childhood trauma and the severe clinical features of BD. METHOD: A total of 342 participants with BD were recruited from France and Norway...
November 29, 2016: Psychological Medicine
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