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https://www.readbyqxmd.com/read/28634653/assessing-the-combined-toxicity-of-bmaa-and-its-isomers-2-4-dab-and-aeg-in-vitro-using-human-neuroblastoma-cells
#1
Brendan J Main, Kenneth J Rodgers
The non-protein amino acid (NPAA) ß-methylamino-L-alanine (BMAA) is produced by a diverse range of cyanobacteria, diatoms and dinoflagellates, and is present in both aquatic and terrestrial ecosystems globally. Exposure to BMAA has been implicated in the development of neurodegenerative diseases including amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD) and Parkinson's disease (PD). BMAA is often found in nature along with its structural isomers 2,4-diaminobutyric acid (2,4-DAB) and aminoethylglycine (AEG); however, the toxicity of these NPAAs in combination has not been examined...
June 20, 2017: Neurotoxicity Research
https://www.readbyqxmd.com/read/28632945/developing-multidisciplinary-clinics-for-neuromuscular-care-and-research
#2
REVIEW
Sabrina Paganoni, Katie Nicholson, Fawn Leigh, Kathryn Swoboda, David Chad, Kristin Drake, Kellen Haley, Merit Cudkowicz, James D Berry
Multidisciplinary care is considered the standard-of-care for both adult and pediatric neuromuscular disorders and has been associated with improved quality of life, resource utilization, and health outcomes. Multidisciplinary care is delivered in multidisciplinary clinics that coordinate care across multiple specialties reducing travel burden and streamlining care. In addition, multidisciplinary care setting facilitates the integration of clinical research, patient advocacy, and care innovation (e.g., TeleHealth)...
June 20, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28632899/novel-intracytoplasmic-inclusions-immunoreactive-for-phosphorylated-tdp43-and-cystatin-c-in-anterior-horn-cells-in-a-case-of-sporadic-amyotrophic-lateral-sclerosis
#3
Masayuki Shintaku, Daita Kaneda, Kiyomitsu Oyanagi
Novel intracytoplasmic inclusions immunoreactive for phosphorylated transactivation response DNA-binding protein 43 (p-TDP43), cystatin C, and transferrin were found in anterior horn cells in a case of sporadic amyotrophic lateral sclerosis (ALS). The patient was a 59-year-old woman, who died of ALS after a clinical course of 8 years. She had been receiving mechanical support for respiration for 6 years and in a "totally locked-in" state for 4 years prior to death. The spinal cord showed severe degeneration involving the anterior and lateral funiculi, whereas the posterior funiculus was preserved...
June 20, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28632852/antioxidant-drug-approved-for-als
#4
Rebecca Voelker
No abstract text is available yet for this article.
June 20, 2017: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/28631959/advance-care-planning-for-patients-with-amyotrophic-lateral-sclerosis
#5
Benjamin H Levi, Zachary Simmons, Courtney Hanna, Allyson Brothers, Erik Lehman, Elana Farace, Megan Bain, Renee Stewart, Michael J Green
PURPOSE: To determine whether an advance care planning (ACP) decision-aid could improve communication about end-of-life treatment wishes between patients with amyotrophic lateral sclerosis (ALS) and their clinicians. METHODS: Forty-four patients with ALS (>21, English-speaking, without dementia) engaged in ACP using an interactive computer based decision-aid. Before participants completed the intervention, and again three months later, their clinicians reviewed three clinical vignettes, and made treatment decisions (n = 18) for patients...
March 5, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28631957/prognostic-value-of-clinical-and-electrodiagnostic-parameters-at-time-of-diagnosis-in-patients-with-amyotrophic-lateral-sclerosis
#6
Wendeline Reniers, Maarten Schrooten, Kristl G Claeys, Petra Tilkin, Ann D'Hondt, Dimphna Van Reijen, Goedele Couwelier, Nikita Lamaire, Wim Robberecht, Steffen Fieuws, Philip Van Damme
OBJECTIVE: To assess the added prognostic value of the aggregated clinical and electrodiagnostic data, which define a given diagnostic category according to the Awaji or revised El Escorial criteria at time of diagnosis in patients with amyotrophic lateral sclerosis (ALS). METHODS: Clinical signs and electrodiagnostic test results were collected at time of diagnosis in 396 patients with ALS between January 2009 and January 2016. Significant predictors of prognosis were identified using a univariate model, and later combined in a multivariate Cox regression model...
February 15, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28631956/coping-strategies-and-psychological-distress-in-caregivers-of-patients-with-amyotrophic-lateral-sclerosis-als
#7
Mattia Siciliano, Gabriella Santangelo, Francesca Trojsi, Carmela Di Somma, Manila Patrone, Cinzia Femiano, Maria Rosaria Monsurrò, Luigi Trojano, Gioacchino Tedeschi
BACKGROUND: Amyotrophic lateral sclerosis (ALS) causes distress in caregivers. The present study aims to examine the association between coping strategies and psychological distress in caregivers of ALS patients. METHODS: Coping strategies were assessed in 96 ALS informal caregivers by means of the Coping Inventory for Stressful Situations. Data about caregivers' demographic characteristics, levels of burden, depression and anxiety (psychological distress) were also gathered by standardised questionnaires...
February 10, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28628797/attenuated-error-related-potentials-in-amyotrophic-lateral-sclerosis-with-executive-dysfunctions
#8
Caroline Seer, Max Joop, Florian Lange, Christian Lange, Reinhard Dengler, Susanne Petri, Bruno Kopp
OBJECTIVE: Executive dysfunctions affect up to 50% of the patients with amyotrophic lateral sclerosis (ALS). Executive dysfunctions have been identified as negative prognostic factor and can affect quality of life in patients and their caregivers. Assessment of executive dysfunction may be impeded by the patients' motor impairments. Event-related potentials (ERP) have been proposed as a tool to overcome these assessment difficulties. The error(-related) negativity (Ne/ERN) is an ERP which can be recorded with minimal motor requirements for the patient...
May 18, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28628244/phosphorylated-neurofilament-heavy-chain-a-biomarker-of-survival-for-c9orf72-associated-amyotrophic-lateral-sclerosis
#9
Tania F Gendron, Lillian M Daughrity, Michael G Heckman, Nancy N Diehl, Joanne Wuu, Timothy M Miller, Pau Pastor, John Q Trojanowski, Murray Grossman, James D Berry, William T Hu, Antonia Ratti, Michael Benatar, Vincenzo Silani, Jonathan D Glass, Mary Kay Floeter, Andreas Jeromin, Kevin B Boylan, Leonard Petrucelli
As potential treatments for C9ORF72-associated amyotrophic lateral sclerosis (c9ALS) approach clinical trials, the identification of prognostic biomarkers for c9ALS becomes a priority. We show that levels of phosphorylated neurofilament heavy chain (pNFH) in cerebrospinal fluid (CSF) predict disease status and survival in c9ALS patients, and are largely stable over time. Moreover, c9ALS patients exhibit higher pNFH levels, more rapid disease progression, and shorter survival after disease onset than ALS patients without C9ORF72 expansions...
June 19, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28627708/alterations-in-aqp4-expression-and-polarization-in-the-course-of-motor-neuron-degeneration-in-sod1g93a-mice
#10
Jiaying Dai, Weihao Lin, Minying Zheng, Qiang Liu, Baixuan He, Chuanming Luo, Xilin Lu, Zhong Pei, Huanxing Su, Xiaoli Yao
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by selective degeneration of upper and lower motor neurons. The disease progression is associated with the astrocytic environment. Aquaporin-4 (AQP4) water channels are the most abundant AQPs expressed in astrocytes, exerting important influences on central nervous system homeostasis. The present study aimed to characterize the alterations in AQP4 expression and loca-lization in superoxide dismutase 1 (SOD1) G93A transgenic mice...
June 15, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28626174/lifestyle-changes-and-oxidative-stress-in-a-high-incidence-area-of-amyotrophic-lateral-sclerosis-in-the-southwestern-kii-peninsula-japan
#11
Tameko Kihira, Kazushi Okamoto, Iori Sakurai, Yuya Arakawa, Ikuro Wakayama, Koichi Takamiya, Ryo Okumura, Yuhto Iinuma, Keiko Iwai, Yasumasa Kokubo, Sohei Yoshida
Objective Lifestyle changes may play an important role in the incidence reduction and delay of onset age of amyotrophic lateral sclerosis (ALS) in the Koza/Kozagawa/Kushimoto (K) area. The aim of this study was to evaluate recent lifestyle changes in the K area and to investigate the relationships between lifestyle and oxidative stress among the residents. Methods We conducted a medical checkup for elderly residents in the K area and the control area and evaluated the urinary 8-OHdG levels, cognitive function test scores and metal contents in serum and scalp hair, coupled with a lifestyle questionnaire survey between 2010 and 2015...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28625609/oxidative-stress-induced-by-cumene-hydroperoxide-produces-synaptic-depression-and-transient-hyperexcitability-in-rat-primary-motor-cortex-neurons
#12
R Pardillo-Diaz, L Carrascal, G Barrionuevo, P Nunez-Abades
Pyramidal neurons of the motor cortex are selectively degenerated in Amyotrophic Lateral Sclerosis (ALS). The mechanisms underlying neuronal death in ALS are not well established. In the absence of useful biomarkers, the early increased neuronal excitability seems to be the unique characteristic of ALS. Lipid peroxidation caused by oxidative stress has been postulated as one of the possible mechanisms involved in degeneration motor cortex pyramidal neurons. This paper examines the effect of lipid peroxidation on layer V pyramidal neurons induced by cumene hydroperoxide (CH) in brain slices from wild type rats...
June 15, 2017: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/28625524/cross-reactivity-and-avidity-of-antibody-responses-induced-in-humans-by-the-oral-inactivated-multivalent-enterotoxigenicescherichia-coli-etec-vaccine-etvax
#13
Susannah Leach, Anna Lundgren, Nils Carlin, Madeleine Löfstrand, Ann-Mari Svennerholm
We investigated whether the oral inactivated, multivalent enterotoxigenic Escherichia coli (ETEC) vaccine ETVAX, consisting of four E. coli strains over-expressing the colonisation factors (CFs) CFA/I, CS3, CS5 and CS6, combined with the toxoid LCTBA, could induce cross-reactive antibodies to CFs related to the CFA/I and CS5 families. We also evaluated the avidity of vaccine induced antibodies against the toxoid and CFs. Cross-reactivity was analysed in mucosal (faecal and antibodies in lymphocyte supernatants, ALS) samples, and antibody avidity in serum and ALS samples, from two phase I trials: a primary vaccination study, where two oral doses of ETVAX were given±the double mutant heat labile toxin (dmLT) adjuvant at a 2-week interval, and a booster vaccination study, where a single booster dose of ETVAX was given 13-23months after primary vaccinations...
June 15, 2017: Vaccine
https://www.readbyqxmd.com/read/28625325/importance-of-cd117-in-the-assignation-of-a-myeloid-lineage-in-acute-leukemias
#14
Alan Pomerantz, Sergio Rodríguez-Rodríguez, Roberta Demichelis-Gómez, Georgina Barrera-Lumbreras, Olga V Barrales-Benítez, María José Díaz-Huízar, Monica Goldberg-Murow, Xavier López-Karpovitch, Álvaro Aguayo
The correct classification of acute leukemias (AL) is an essential part in the evaluation of any patient with this disease. Historically, CD117 has been an important asset in the diagnosis of patients with mixed-phenotype acute leukemia (MPAL). In an attempt to simplify the diagnosis of MPAL with fewer and more lineage specific markers, the World Health Organization (WHO) proposed in 2008 a new criteria for the diagnosis of this type of AL, which excluded CD117 from the myeloid markers that are utilized to diagnose MPAL...
February 2017: Archives of Medical Research
https://www.readbyqxmd.com/read/28624594/variability-in-the-initiation-of-resuscitation-attempts-by-emergency-medical-services-personnel-during-out-of-hospital-cardiac-arrest
#15
Steven C Brooks, Robert H Schmicker, Sheldon Cheskes, James Christenson, Alan Craig, Mohamud Daya, Peter J Kudenchuk, Graham Nichol, Dana Zive, Laurie J Morrison
BACKGROUND: Some patients with out-of-hospital cardiac arrest (OHCA) assessed by emergency medical services (EMS) do not receive attempts at resuscitation on the basis of perceived futility. AIMS: 1) To measure variability in the initiation of resuscitation attempts in EMS-assessed OHCA patients across EMS agencies, 2) to evaluate the association between selected EMS agency characteristics and the proportion of patients receiving resuscitation attempts, and 3) to evaluate the association between proportion receiving resuscitation attempts and survival...
June 14, 2017: Resuscitation
https://www.readbyqxmd.com/read/28624492/cortical-function-and-corticomotoneuronal-adaptation-in-monomelic-amyotrophy
#16
José Manuel Matamala, Nimeshan Geevasinga, William Huynh, Thanuja Dharmadasa, James Howells, Neil G Simon, Parvathi Menon, Steve Vucic, Matthew C Kiernan
OBJECTIVE: To evaluate corticomotoneuronal integrity in monomelic amyotrophy using threshold tracking transcranial magnetic stimulation (TT-TMS). METHODS: Cortical excitability studies were prospectively performed in 8 monomelic amyotrophy patients and compared to 21 early-onset amyotrophic lateral sclerosis (ALS) patients and 40 healthy controls. Motor evoked potentials responses were recorded over abductor pollicis brevis. RESULTS: Maximal motor evoked potential (MEP/CMAP ratio) was significantly increased in monomelic amyotrophy compared with controls (monomelic amyotrophy 51...
May 17, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28624261/dutch-health-websites-and-their-ability-to-inform-people-with-low-health-literacy
#17
Corine S Meppelink, Julia C M van Weert, Anna Brosius, Edith G Smit
OBJECTIVE: To evaluate whether Dutch online health information (OHI) generally reflects message elements that support information processing and understanding among people with low health literacy. METHODS: We content-analyzed one hundred Dutch webpages about Ebola, fibromyalgia, ALS, losing weight, borderline personality disorder, hemorrhoids, ADD, bladder infection, shingles, and chicken pox. The codebook covered the following domains: images and videos, readability level, Suitability Assessment of Materials (SAM), advertising, interactive features, and reliability cues...
June 10, 2017: Patient Education and Counseling
https://www.readbyqxmd.com/read/28623007/gene-networks-in-neurodegenerative-disorders
#18
REVIEW
Daniela Recabarren, Marcelo Alarcón
Three neurodegenerative diseases [Amyotrophic Lateral Sclerosis (ALS), Parkinson's disease (PD) and Alzheimer's disease (AD)] have many characteristics like pathological mechanisms and genes. In this sense some researchers postulate that these diseases share the same alterations and that one alteration in a specific protein triggers one of these diseases. Analyses of gene expression may shed more light on how to discover pathways, pathologic mechanisms associated with the disease, biomarkers and potential therapeutic targets...
June 13, 2017: Life Sciences
https://www.readbyqxmd.com/read/28622300/the-als-linked-e102q-mutation-in-sigma-receptor-1-leads-to-er-stress-mediated-defects-in-protein-homeostasis-and-dysregulation-of-rna-binding-proteins
#19
Alice Dreser, Jan Tilmann Vollrath, Antonio Sechi, Sonja Johann, Andreas Roos, Alfred Yamoah, Istvan Katona, Saeed Bohlega, Dominik Wiemuth, Yuemin Tian, Axel Schmidt, Jörg Vervoorts, Marc Dohmen, Cordian Beyer, Jasper Anink, Eleonora Aronica, Dirk Troost, Joachim Weis, Anand Goswami
Amyotrophic lateral sclerosis (ALS) is characterized by the selective degeneration of motor neurons (MNs) and their target muscles. Misfolded proteins which often form intracellular aggregates are a pathological hallmark of ALS. Disruption of the functional interplay between protein degradation (ubiquitin proteasome system and autophagy) and RNA-binding protein homeostasis has recently been suggested as an integrated model that merges several ALS-associated proteins into a common pathophysiological pathway...
June 16, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/28621357/computational-investigation-of-the-human-sod1-mutant-cys146arg-that-directs-familial-amyotrophic-lateral-sclerosis
#20
E Srinivasan, R Rajasekaran
The genetic substitution mutation of Cys146Arg in the SOD1 protein is predominantly found in the Japanese population suffering from familial amyotrophic lateral sclerosis (FALS). A complete study of the biophysical aspects of this particular missense mutation through conformational analysis and producing free energy landscapes could provide an insight into the pathogenic mechanism of ALS disease. In this study, we utilized general molecular dynamics simulations along with computational predictions to assess the structural characterization of the protein as well as the conformational preferences of monomeric wild type and mutant SOD1...
June 16, 2017: Molecular BioSystems
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