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https://www.readbyqxmd.com/read/29331073/cerebrospinal-fluid-macrophage-biomarkers-in-amyotrophic-lateral-sclerosis
#1
A G Thompson, E Gray, M-L Thézénas, P D Charles, S Evetts, M T Hu, K Talbot, R Fischer, B M Kessler, M R Turner
Objective The neurodegenerative disease amyotrophic lateral sclerosis (ALS) is a heterogeneous clinical syndrome involving multiple molecular pathways. The development of biomarkers for use in therapeutic trials is a priority. We sought to use a high-throughput proteomic method to identify novel biomarkers in individual cerebrospinal fluid samples. Methods Liquid chromatography-tandem mass spectrometry with label-free quantification was used to identify cerebrospinal fluid proteins using samples from a well-characterised longitudinal cohort comprising patients with ALS (n=43), the upper motor neuron variant primary lateral sclerosis (PLS, n=6), cross-sectional healthy (n=20) and disease controls (Parkinsons's n=20, ALS mimic disorders n=12)...
January 13, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29324454/using-the-capture-recapture-method-to-estimate-the-incidence-of-amyotrophic-lateral-sclerosis-in-beijing-china
#2
Shenghan Zhou, Silin Qian, Xiaohan Li, Liping Zheng, Wenbing Chang, Liping Wang
BACKGROUND: To assess the total, gender-related and ageing process-related incidence rates of amyotrophic lateral sclerosis (ALS) in Beijing, China. Determine whether the decreased male to female ratio among postmenopausal age groups. METHODS: We used the 2-source capture-recapture method to estimate the incidence of ALS in Beijing. The primary and secondary data sources were from diagnostic hospitals and assisted care institutions in the same area from 2010 to 2015...
January 11, 2018: Neuroepidemiology
https://www.readbyqxmd.com/read/29322304/causative-genes-in-amyotrophic-lateral-sclerosis-and-protein-degradation-pathways-a-link-to-neurodegeneration
#3
REVIEW
C Maurel, A Dangoumau, S Marouillat, C Brulard, A Chami, R Hergesheimer, P Corcia, H Blasco, C R Andres, P Vourc'h
Amyotrophic lateral sclerosis (ALS) is a disease caused by the degeneration of motor neurons (MNs) leading to progressive muscle weakness and atrophy. Several molecular pathways have been implicated, such as glutamate-mediated excitotoxicity, defects in cytoskeletal dynamics and axonal transport, disruption of RNA metabolism, and impairments in proteostasis. ALS is associated with protein accumulation in the cytoplasm of cells undergoing neurodegeneration, which is a hallmark of the disease. In this review, we focus on mechanisms of proteostasis, particularly protein degradation, and discuss how they are related to the genetics of ALS...
January 10, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29321969/the-two-year-progression-of-structural-and-functional-cerebral-mri-in-amyotrophic-lateral-sclerosis
#4
R A L Menke, M Proudfoot, K Talbot, M R Turner
MRI has emerged as one of several urgently needed candidate disease progression biomarkers for the neurodegenerative disorder amyotrophic lateral sclerosis (ALS), not least due to its unique ability to non-invasively assess structural and functional cerebral pathology. We sought to identify the extent of detectable change in cerebral MRI metrics over a more prolonged period. Analysis of multi-modal MRI data was performed in a cohort of sixteen patients (13 ALS and 3 with primary lateral sclerosis) in whom it was possible to acquire six-monthly images over two years...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29317192/abnormal-cortical-brain-integration-of-somatosensory-afferents-in-als
#5
Sina Sangari, Alain Giron, Guillaume Marrelec, Pierre-François Pradat, Véronique Marchand-Pauvert
OBJECTIVES: Infraclinical sensory alterations have been reported at early stages of amyotrophic lateral sclerosis (ALS). While previous studies mainly focused on early somatosensory evoked potentials (SEPs), late SEPs, which reflect on cortical pathways involved in cognitive-motor functions, are relatively underinvestigated. Early and late SEPs were compared to assess their alterations in ALS. METHODS: Median and ulnar nerves were electrically stimulated at the wrist, at 9 times the perceptual threshold, in 21 ALS patients without clinical evidence of sensory deficits, and 21 age- and gender-matched controls...
December 24, 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29316850/demographics-and-clinical-characteristics-of-primary-lateral-sclerosis-case-series-and-a-review-of-literature
#6
Ramnath Santosh Ramanathan, Sandeep Rana
AIM: Primary lateral sclerosis (PLS) is a form of motor neuron disease involving only upper motor neurons. In some patients presenting as PLS, the disease progresses to involve lower motor neurons and thereby converting to amyotrophic lateral sclerosis (ALS). However, pure forms of PLS do exist. Our aim was to study epidemiological and clinical characteristics of pure PLS patients treated at our neuromuscular clinic. METHODS: We retrospectively reviewed 15 patients from July 2011 to October 2014 with PLS treated at the neuromuscular disorder clinic at our hospital...
January 10, 2018: Neurodegenerative Disease Management
https://www.readbyqxmd.com/read/29314106/toenail-mercury-levels-are-associated-with-amyotrophic-lateral-sclerosis-als-risk
#7
Angeline S Andrew, Celia Y Chen, Tracie A Caller, Rup Tandan, Patricia L Henegan, Brian P Jackson, Brenda P Hall, Walter G Bradley, Elijah W Stommel
INTRODUCTION: Mercury is a neurotoxic metal that is potentially a risk factor for amyotrophic lateral sclerosis (ALS). Consumption of methylmercury contaminated fish is the primary source of U.S. population exposure to mercury. METHODS: We used inductively coupled plasma mass spectrometry to measure levels of mercury in toenail samples from ALS patients (n=46) and from controls (n=66), as a biomarker of mercury exposure. RESULTS: ALS patients had higher toenail mercury levels (OR 2...
January 4, 2018: Muscle & Nerve
https://www.readbyqxmd.com/read/29311743/tdp-43-pathology-disrupts-nuclear-pore-complexes-and-nucleocytoplasmic-transport-in-als-ftd
#8
Ching-Chieh Chou, Yi Zhang, Mfon E Umoh, Spencer W Vaughan, Ileana Lorenzini, Feilin Liu, Melissa Sayegh, Paul G Donlin-Asp, Yu Han Chen, Duc M Duong, Nicholas T Seyfried, Maureen A Powers, Thomas Kukar, Chadwick M Hales, Marla Gearing, Nigel J Cairns, Kevin B Boylan, Dennis W Dickson, Rosa Rademakers, Yong-Jie Zhang, Leonard Petrucelli, Rita Sattler, Daniela C Zarnescu, Jonathan D Glass, Wilfried Rossoll
The cytoplasmic mislocalization and aggregation of TAR DNA-binding protein-43 (TDP-43) is a common histopathological hallmark of the amyotrophic lateral sclerosis and frontotemporal dementia disease spectrum (ALS/FTD). However, the composition of aggregates and their contribution to the disease process remain unknown. Here we used proximity-dependent biotin identification (BioID) to interrogate the interactome of detergent-insoluble TDP-43 aggregates and found them enriched for components of the nuclear pore complex and nucleocytoplasmic transport machinery...
January 8, 2018: Nature Neuroscience
https://www.readbyqxmd.com/read/29308690/overexpression-of-a-conserved-hsp40-chaperone-reduces-toxicity-of-several-neurodegenerative-disease-proteins
#9
Sei-Kyoung Park, Fatih Arslan, Vydehi Kanneganti, Sami J Barmada, Pravinkumar Purushothaman, Subhash Chandra Verma, Susan W Liebman
TDP-43 and FUS are DNA/RNA binding proteins associated with neuronal inclusions in amyotrophic lateral sclerosis (ALS) patients. Other neurodegenerative diseases are also characterized by neuronal protein aggregates, e.g. Huntington's disease, associated with polyglutamine (polyQ) expansions in the protein huntingtin. Here we discuss our recent paper establishing similarities between aggregates of TDP-43 that have short glutamine and asparagine (Q/N)-rich modules and are soluble in detergents, with those of polyQ and PIN4C that have large Q/N-rich domains and are detergent-insoluble...
January 8, 2018: Prion
https://www.readbyqxmd.com/read/29308669/an-open-label-study-of-a-novel-immunosuppression-intervention-for-the-treatment-of-amyotrophic-lateral-sclerosis
#10
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...
January 8, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29303786/aerobic-glycolysis-in-amyotrophic-lateral-sclerosis-and-huntington-s-disease
#11
REVIEW
Alexandre Vallée, Yves Lecarpentier, Rémy Guillevin, Jean-Noël Vallée
Neurodegenerative cells are the sites of numerous metabolic and energetic abnormalities with abnormalities in energy production. Energy is the primary determinant of neuronal viability. In neurodegenerative cells, metabolic enzymes are modified by the dysregulation of the canonical WNT/β-catenin pathway. In amyotrophic lateral sclerosis (ALS) and Huntington's disease (HD), WNT/β-catenin pathway is upregulated. We focused this review on the hypothesis of aerobic glycolysis stimulated by the upregulation of WNT/β-catenin pathway in ALS and HD...
January 5, 2018: Reviews in the Neurosciences
https://www.readbyqxmd.com/read/29302060/c9orf72-ggggcc-repeat-associated-non-aug-translation-is-upregulated-by-stress-through-eif2%C3%AE-phosphorylation
#12
Weiwei Cheng, Shaopeng Wang, Alexander A Mestre, Chenglai Fu, Andres Makarem, Fengfan Xian, Lindsey R Hayes, Rodrigo Lopez-Gonzalez, Kevin Drenner, Jie Jiang, Don W Cleveland, Shuying Sun
Hexanucleotide repeat expansion in C9ORF72 is the most frequent cause of both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Here we demonstrate that the repeat-associated non-AUG (RAN) translation of (GGGGCC) n -containing RNAs into poly-dipeptides can initiate in vivo without a 5'-cap. The primary RNA substrate for RAN translation of C9ORF72 sense repeats is shown to be the spliced first intron, following its excision from the initial pre-mRNA and transport to the cytoplasm. Cap-independent RAN translation is shown to be upregulated by various stress stimuli through phosphorylation of the α subunit of eukaryotic initiation factor-2 (eIF2α), the core event of an integrated stress response (ISR)...
January 4, 2018: Nature Communications
https://www.readbyqxmd.com/read/29284602/cervical-cord-atrophy-and-long-term-disease-progression-in-patients-with-primary-progressive-multiple-sclerosis
#13
F X Aymerich, C Auger, J Alonso, M Alberich, J Sastre-Garriga, M Tintoré, X Montalban, A Rovira
BACKGROUND AND PURPOSE: Cervical cord atrophy has been associated with clinical disability in multiple sclerosis and is proposed as an outcome measure of neurodegeneration. The aim of this study was to quantify the development of cervical cord atrophy and to evaluate its association with disability progression in patients with primary-progressive multiple sclerosis. MATERIALS AND METHODS: Thirty-one patients with primary-progressive multiple sclerosis underwent 1...
December 28, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29278899/the-utility-of-the-laboratory-work-up-at-the-time-of-diagnosis-of-amyotrophic-lateral-sclerosis
#14
Ario Mirian, Lawrence Korngut
BACKGROUND: Serological testing is routinely performed in the work up for a diagnosis of Amyotrophic Lateral Sclerosis (ALS) to exclude pathologies with similar clinical phenotypes. OBJECTIVE: To determine the proportion of serological workup that changes the primary diagnosis and/or clinical management for patients presenting with signs of ALS. METHODS: A retrospective chart review was conducted on patients from the Calgary Neuromuscular Intake Clinic in which the neurologist working diagnosis post-assessment is ALS...
December 20, 2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/29260014/regional-glucose-hypometabolic-spread-within-the-primary-motor-cortex-is-associated-with-amyotrophic-lateral-sclerosis-disease-progression-a-fluoro-deoxyglucose-positron-emission-tomography-study
#15
Hironobu Endo, Kenji Sekiguchi, Takehiro Ueda, Hisatomo Kowa, Fumio Kanda, Tatsushi Toda
Objective: Here we investigate the process of neurodegeneration in amyotrophic lateral sclerosis (ALS). The relationship between the cortical field spreading of glucose metabolic decreases in the primary motor cortex (PMC) and the progression of corresponding extremity dysfunction was evaluated using [18F] fluoro-deoxyglucose (FDG)-positron emission tomography (PET). Methods: Patients with ALS underwent [18F] FDG-PET and the resulting datasets were individually contrasted against healthy controls using the NEUROSTAT software...
March 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29226778/a-multicenter-randomized-controlled-trial-of-two-group-education-programs-for-fatigue-in-multiple-sclerosis-short-and-medium-term-benefits
#16
Cinda L Hugos, Zunqiu Chen, Yiyi Chen, Aaron P Turner, Jodie Haselkorn, Toni Chiara, Sean McCoy, Christopher T Bever, Michelle H Cameron, Dennis Bourdette
Background Fatigue occurs in 75%-95% of people with multiple sclerosis (MS) and is frequently reported as the most disabling symptom. A multicomponent group program of six weekly 2-hour sessions, Fatigue: Take Control (FTC), was developed from an international MS fatigue management guideline. Objective To determine whether FTC is associated with greater improvements in fatigue than MS: Take Control (MSTC), a similarly structured general MS education program. Methods This four-site, parallel, single-blind, randomized controlled trial compared FTC and MSTC in 204 ambulatory participants with MS...
December 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/29207498/steppogenin-isolated-from-cudrania-tricuspidata-shows-antineuroinflammatory-effects-via-nf-%C3%AE%C2%BAb-and-mapk-pathways-in-lps-stimulated-bv2-and-primary-rat-microglial-cells
#17
Dong-Cheol Kim, Tran Hong Quang, Hyuncheol Oh, Youn-Chul Kim
Excessive microglial stimulation has been recognized in several neurodegenerative diseases, including Parkinson's disease (PD), Alzheimer's disease (AD), amyotropic lateral sclerosis (ALS), HIV-associated dementia (HAD), multiple sclerosis (MS), and stroke. When microglia are stimulated, they produce proinflammatory mediators and cytokines, including nitric oxide (NO) derived from inducible NO synthase (iNOS), prostaglandin E2 (PGE₂) derived from cyclooxygenase-2 (COX-2), tumor necrosis factor-α (TNF-α), interleukin-1β (IL-1β), interleukin-12 (IL-12), and interleukin-6 (IL-6)...
December 2, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29194436/characterisation-of-the-pathogenic-effects-of-the-in-vivo-expression-of-an-als-linked-mutation-in-d-amino-acid-oxidase-phenotype-and-loss-of-spinal-cord-motor-neurons
#18
Nazanin Rahmani Kondori, Praveen Paul, Jacqueline P Robbins, Ke Liu, John C W Hildyard, Dominic J Wells, Jacqueline S de Belleroche
Amyotrophic lateral sclerosis (ALS) is the most common adult-onset neuromuscular disorder characterised by selective loss of motor neurons leading to fatal paralysis. Current therapeutic approaches are limited in their effectiveness. Substantial advances in understanding ALS disease mechanisms has come from the identification of pathogenic mutations in dominantly inherited familial ALS (FALS). We previously reported a coding mutation in D-amino acid oxidase (DAOR199W) associated with FALS. DAO metabolises D-serine, an essential co-agonist at the N-Methyl-D-aspartic acid glutamate receptor subtype (NMDAR)...
2017: PloS One
https://www.readbyqxmd.com/read/29194293/multiple-pathological-mechanisms-contribute-to-hippocampal-damage-in-the-experimental-autoimmune-encephalomyelitis-model-of-multiple-sclerosis
#19
Elizabeth L Kyran, Christine Robinson, Pece Kocovski, Zhenjiang Li, Phuc T Dang, Matthew W Hale, Jacqueline M Orian
Emotional and cognitive deficits and associated hippocampal damage observed in multiple sclerosis (MS) are now recognized as primary disease manifestations. However, the pathological substrate of these dysfunctions is unclear. In the experimental autoimmune encephalomyelitis (EAE) MS model, impaired hippocampal-dependent functions are concomitant with severe microglial reactivity and neurodegeneration, but reports vary with respect to evidence of lymphocytic infiltration, raising questions as to the nature of the underlying neurodegenerative mechanisms...
January 3, 2018: Neuroreport
https://www.readbyqxmd.com/read/29191052/a-randomized-controlled-trial-of-resistance-and-endurance-exercise-in-amyotrophic-lateral-sclerosis
#20
Lora L Clawson, Merit Cudkowicz, Lisa Krivickas, Benjamin R Brooks, Mohammed Sanjak, Peggy Allred, Nazem Atassi, Amy Swartz, Gabrielle Steinhorn, Alpa Uchil, Kristen M Riley, Hong Yu, David A Schoenfeld, Nicholas J Maragakis
OBJECTIVE: Evaluate the safety and tolerability of resistance and endurance exercise in ALS participants as measured by their ability to complete this six-month study. METHODS: Participants were randomized to Resistance, Endurance, or Stretching/Range of Motion (SROM the exercise regimen prescribed for most ALS patients) exercises. All exercises were performed at home with an individualized regimen designed by a physical therapist trained in ALS management. Primary outcome measures were tolerability of the exercises at 24 weeks defined by 50% of participants completing at least 50% of the prescribed exercise regimen...
November 30, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
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