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https://www.readbyqxmd.com/read/27915482/correlation-between-forced-vital-capacity-and-slow-vital-capacity-for-the-assessment-of-respiratory-involvement-in-amyotrophic-lateral-sclerosis-a-prospective-study
#1
Susana Pinto, Mamede de Carvalho
INTRODUCTION: Slow vital capacity (SVC) and forced vital capacity (FVC) are the most frequent used tests evaluating respiratory function in amyotrophic lateral sclerosis (ALS). No previous study has determined their interchangeability. OBJECTIVE: To evaluate SVC-FVC correlation in ALS. METHODS: Consecutive definite/probable ALS and primary lateral sclerosis (PLS) patients (2000-2014) in whom respiratory tests were performed at baseline/4-6months later were included...
December 4, 2016: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/27915116/7t-mr-of-intracranial-pathology-preliminary-observations-and-comparisons-to-3t-and-1-5t
#2
Emmanuel C Obusez, Mark Lowe, Se-Hong Oh, Irene Wang, M S Jennifer Bullen, Paul Ruggieri, Virginia Hill, Daniel Lockwood, Todd Emch, Doksu Moon, Gareth Loy, Jonathan Lee, Matthew Kiczek, D O Manoj Massand, Volodymyr Statsevych, Todd Stultz, Stephen E Jones
PURPOSE: There have been an increasing number of studies involving ultra-high-field 7T of intracranial pathology, however, comprehensive clinical studies of neuropathology at 7T still remain limited. 7T has the advantage of a higher signal-to-noise ratio and a higher contrast-to-noise ratio, compared to current low field clinical MR scanners. We hypothesized 7T applied clinically, may improve detection and characterization of intracranial pathology. MATERIALS AND METHODS: We performed an IRB-approved 7T prospective study of patients with neurological disease who previously had lower field 3T and 1...
November 30, 2016: NeuroImage
https://www.readbyqxmd.com/read/27904119/an-autopsy-case-of-amyotrophic-lateral-sclerosis-with-diaphragm-pacing
#3
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
#4
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/27880990/neurodegeneration-and-nlrp3-inflammasome-expression-in-the-anterior-thalamus-of-sod1-g93a-als-mice
#5
Berthold Debye, Lena Schmülling, Lepu Zhou, Gabriele Rune, Cordian Beyer, Sonja Johann
Nowadays, Amyotrophic lateral sclerosis (ALS) is considered as a multisystem disorder, characterized by a primary degeneration of motor neurons as well as neuropathological changes in non-motor regions. Neurodegeneration in subcortical areas, such as the thalamus, are believed to contribute to cognitive and behavioural abnormalities in ALS patients. In the present study, we investigated neurodegenerative changes including neuronal loss and glia pathology in the anterodorsal thalamic nucleus (AD) of SOD1(G93A) mice, a widely used animal model for ALS...
November 23, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27860375/differential-impairment-of-thalamocortical-structural-connectivity-in-amyotrophic-lateral-sclerosis
#6
Jiu-Quan Zhang, Bing Ji, Chao-Yang Zhou, Long-Chuan Li, Zhi-Hao Li, Xiao-Ping Hu, Jun Hu
AIMS: The thalamus is a major relay station that modulates input from many cortical areas and a filter for sensory input and is involved in the pathophysiology of amyotrophic lateral sclerosis (ALS). However, it still remains unclear whether all thalamocortical networks are affected or whether there is selective vulnerability. In this study, we aimed to study the selective vulnerability of different thalamocortical structural connections in ALS and to test the hypothesis of a specific impairment in motor-related thalamocortical connectivity...
November 12, 2016: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/27858953/the-concept-and-diagnostic-criteria-of-primary-lateral-sclerosis
#7
Verena Wais, Angela Rosenbohm, Susanne Petri, Katja Kollewe, Andreas Hermann, Alexander Storch, Frank Hanisch, Stephan Zierz, Gabriele Nagel, Jan Kassubek, Patrick Weydt, Johannes Brettschneider, Jochen H Weishaupt, Albert C Ludolph, Johannes Dorst
OBJECTIVES: Primary lateral sclerosis (PLS) is commonly considered as a motor neuron disease (MND) variant which almost exclusively affects upper motor neurons (UMN). There is still no consensus whether PLS should be regarded as an independent disease entity separate from amyotrophic lateral sclerosis (ALS) or as a comparatively slowly progressive variant of ALS. Given these different views, clinical diagnosis of PLS is a challenge. In this multicenter study, we analyzed clinical features of patients diagnosed with PLS in four specialized MND centers...
November 15, 2016: Acta Neurologica Scandinavica
https://www.readbyqxmd.com/read/27823929/expression-of-secreted-phosphoprotein-1-osteopontin-in-human-sensorimotor-cortex-and-spinal-cord-changes-in-patients-with-amyotrophic-lateral-sclerosis
#8
Tatsuya Yamamoto, Shigeo Murayama, Masaki Takao, Tadashi Isa, Noriyuki Higo
Secreted phosphoprotein 1 (SPP1, also known as osteopontin) is expressed in large pyramidal neurons in the primary motor cortex (M1) of certain primate species, including macaque monkeys, but not of rodents. Based on this, we suggested that SPP1 expression may reflect the functional or structural specialization of highly developed corticospinal systems. In the present study, we further characterized SPP1 in the human central nervous system by investigating its expression in the primary somatosensory cortex (S1) and spinal cord, in addition to M1...
November 5, 2016: Brain Research
https://www.readbyqxmd.com/read/27822184/long-term-use-of-riluzole-could-improve-the-prognosis-of-sporadic-amyotrophic-lateral-sclerosis-patients-a-real-world-cohort-study-in-china
#9
Lu Chen, Xiaolu Liu, Lu Tang, Nan Zhang, Dongsheng Fan
Objectives: To investigate the effectiveness of riluzole in a long-term follow-up of cohort with sporadic amyotrophic lateral sclerosis (ALS) in a real-world study. Methods: Patients with ALS between 2007 and 2013 were followed up every 3 months. Survival and tracheotomy were predefined as primary outcome measures. The cumulative defined daily dose (cDDD) of riluzole was estimated. The patients in the riluzole group were classified into 1 of 3 subgroups according to the cDDD quartiles. Survival was analyzed using Kaplan-Meier and Cox regression analysis...
2016: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/27818323/als-causing-mutations-differentially-affect-pgc-1%C3%AE-expression-and-function-in-the-brain-vs-peripheral-tissues
#10
Hanna Bayer, Kerstin Lang, Eva Buck, Julia Higelin, Lara Barteczko, Noemi Pasquarelli, Jasmin Sprissler, Tanja Lucas, Karlheinz Holzmann, Maria Demestre, Katrin S Lindenberg, Karin M Danzer, Tobias Boeckers, Albert C Ludolph, Luc Dupuis, Patrick Weydt, Anke Witting
BACKGROUND: Monogenetic forms of amyotrophic lateral sclerosis (ALS) offer an opportunity for unraveling the molecular mechanisms underlying this devastating neurodegenerative disorder. In order to identify a link between ALS-related metabolic changes and neurodegeneration, we investigated whether ALS-causing mutations interfere with the peripheral and brain-specific expression and signaling of the metabolic master regulator PGC (PPAR gamma coactivator)-1α (PGC-1α). METHODS: We analyzed the expression of PGC-1α isoforms and target genes in two mouse models of familial ALS and validated the stimulated PGC-1α signaling in primary adipocytes and neurons of these animal models and in iPS derived motoneurons of two ALS patients harboring two different frame-shift FUS/TLS mutations...
November 3, 2016: Neurobiology of Disease
https://www.readbyqxmd.com/read/27793099/severe-respiratory-changes-at-end-stage-in-a-fus-induced-disease-state-in-adult-rats
#11
Kasey L Jackson, Hemangini A Dhaibar, Robert D Dayton, Sergio G Cananzi, William G Mayhan, Edward Glasscock, Ronald L Klein
BACKGROUND: Fused in sarcoma (FUS) is an RNA-binding protein associated with the neurodegenerative diseases amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. ALS manifests in patients as a progressive paralysis which leads to respiratory dysfunction and failure, the primary cause of death in ALS. We expressed human FUS in rats to determine if FUS would induce ALS relevant respiratory changes to serve as an early stage disease indicator. The FUS expression was initiated in adult rats by way of an intravenously administered adeno-associated virus vector serotype 9 (AAV9) providing an adult onset model...
October 28, 2016: BMC Neuroscience
https://www.readbyqxmd.com/read/27790792/als-associated-endoplasmic-reticulum-proteins-in-denervated-skeletal-muscle-implications-for-motor-neuron-disease-pathology
#12
C M Jesse, E Bushuven, P Tripathi, A Chandrasekar, C M Simon, C Drepper, A Yamoah, A Dreser, I Katona, S Johann, C Beyer, S Wagner, M Grond, S Nikolin, J Anink, D Troost, M Sendtner, A Goswami, J Weis
Alpha-motoneurons and muscle fibers are structurally and functionally interdependent. Both cell types particularly rely on endoplasmic reticulum (ER/SR) functions. Mutations of the ER proteins VAPB, SigR1 and HSP27 lead to hereditary motor neuron diseases (MNDs). Here, we determined the expression profile and localization of these ER proteins/chaperons by immunohistochemistry and immunoblotting in biopsy and autopsy muscle tissue of patients with amyotrophic lateral sclerosis (ALS) and other neurogenic muscular atrophies (NMAs) and compared these patterns to mouse models of neurogenic muscular atrophy...
October 28, 2016: Brain Pathology
https://www.readbyqxmd.com/read/27776165/distinct-c9orf72-associated-dipeptide-repeat-structures-correlate-with-neuronal-toxicity
#13
Brittany N Flores, Mark E Dulchavsky, Amy Krans, Michael R Sawaya, Henry L Paulson, Peter K Todd, Sami J Barmada, Magdalena I Ivanova
Hexanucleotide repeat expansions in C9orf72 are the most common inherited cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The expansions elicit toxicity in part through repeat-associated non-AUG (RAN) translation of the intronic (GGGGCC)n sequence into dipeptide repeat-containing proteins (DPRs). Little is known, however, about the structural characteristics and aggregation propensities of the dipeptide units comprising DPRs. To address this question, we synthesized dipeptide units corresponding to the three sense-strand RAN translation products, analyzed their structures by circular dichroism, electron microscopy and dye binding assays, and assessed their relative toxicity when applied to primary cortical neurons...
2016: PloS One
https://www.readbyqxmd.com/read/27768524/c9orf72-plays-a-central-role-in-rab-gtpase-dependent-regulation-of-autophagy
#14
Christopher P Webster, Emma F Smith, Andrew J Grierson, Kurt J De Vos
A GGGGCC hexanucleotide repeat expansion in the first intron of the C9orf72 gene is the most common genetic defect associated with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) (C9ALS/FTD). Haploinsufficiency and a resulting loss of C9orf72 protein function has been suggested as a possible pathogenic mechanism in C9ALS/FTD. C9ALS/FTD patients exhibit specific ubiquitin and p62/sequestosome-1 positive but TDP-43 negative inclusions in the cerebellum and hippocampus, indicating possible autophagy deficits in these patients...
October 21, 2016: Small GTPases
https://www.readbyqxmd.com/read/27757524/pathological-tdp-43-changes-in-betz-cells-differ-from-those-in-bulbar-and-spinal-%C3%AE-motoneurons-in-sporadic-amyotrophic-lateral-sclerosis
#15
Heiko Braak, Albert C Ludolph, Manuela Neumann, John Ravits, Kelly Del Tredici
Two nerve cells types, Betz cells in layer Vb of the primary motor neocortex and α-motoneurons of the lower brainstem and spinal cord, become involved at the beginning of the pathological cascade underlying sporadic amyotrophic lateral sclerosis (sALS). In both neuronal types, the cell nuclei forfeit their normal (non-phosphorylated) expression of the 43-kDa transactive response DNA-binding protein (TDP-43). Here, we present initial evidence that in α-motoneurons the loss of normal nuclear TDP-43 expression is followed by the formation of phosphorylated TDP-43 aggregates (pTDP-43) within the cytoplasm, whereas in Betz cells, by contrast, the loss of normal nuclear TDP-43 expression remains mostly unaccompanied by the development of cytoplasmic aggregations...
October 18, 2016: Acta Neuropathologica
https://www.readbyqxmd.com/read/27756805/widespread-structural-brain-involvement-in-als-is-not-limited-to-the-c9orf72-repeat-expansion
#16
Henk-Jan Westeneng, Renée Walhout, Milou Straathof, Ruben Schmidt, Jeroen Hendrikse, Jan H Veldink, Martijn P van den Heuvel, Leonard H van den Berg
BACKGROUND: In patients with a C9orf72 repeat expansion (C9+), a neuroimaging phenotype with widespread structural cerebral changes has been found. We aimed to investigate the specificity of this neuroimaging phenotype in patients with amyotrophic lateral sclerosis (ALS). METHODS: 156 C9- and 14 C9+ patients with ALS underwent high-resolution T1-weighted MRI; a subset (n=126) underwent diffusion-weighted imaging. Cortical thickness, subcortical volumes and white matter integrity were compared between C9+ and C9- patients...
October 18, 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/27751553/early-diaphragm-pacing-in-patients-with-amyotrophic-lateral-sclerosis-respistimals-a-randomised-controlled-triple-blind-trial
#17
Jésus Gonzalez-Bermejo, Capucine Morélot-Panzini, Marie-Laure Tanguy, Vincent Meininger, Pierre-François Pradat, Timothée Lenglet, Gaëlle Bruneteau, Nadine Le Forestier, Philippe Couratier, Nathalie Guy, Claude Desnuelle, Hélène Prigent, Christophe Perrin, Valérie Attali, Catherine Fargeot, Marie-Cécile Nierat, Catherine Royer, Fabrice Ménégaux, François Salachas, Thomas Similowski
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder associated with respiratory muscle weakness and respiratory failure. Non-invasive ventilation alleviates respiratory symptoms and prolongs life, but is a palliative intervention. Slowing the deterioration of diaphragm function before respiratory failure would be desirable. We aimed to assess whether early diaphragm pacing could slow down diaphragm deterioration and would therefore delay the need for non-invasive ventilation...
November 2016: Lancet Neurology
https://www.readbyqxmd.com/read/27699797/intermuscular-coherence-in-als-a-preliminary-assessment
#18
Naoum P Issa, Samuel Frank, Raymond Roos, Betty Soliven, Vernon L Towle, Kourosh Rezania
INTRODUCTION: Simple laboratory tests of upper motor neuron involvement in amyotrophic lateral sclerosis (ALS) are not available. Intermuscular coherence has been shown to distinguish patients with primary lateral sclerosis, a pure upper motor neuron disorder, from normal subjects, suggesting it could be useful for assessing ALS. OBJECTIVE: To determine whether intermuscular coherence can distinguish ALS patients from normal subjects. METHODS: We measured biceps brachii and brachioradialis activity using surface electromyography while subjects held the elbow at flexion and the forearm in semi-pronation...
October 3, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27694488/identification-and-outcomes-of-clinical-phenotypes-in-amyotrophic-lateral-sclerosis-motor-neuron-disease-australian-national-motor-neuron-disease-observational-cohort
#19
Paul Talman, Thi Duong, Steve Vucic, Susan Mathers, Svetha Venkatesh, Robert Henderson, Dominic Rowe, David Schultz, Robert Edis, Merrilee Needham, Richard Macdonnell, Pamela McCombe, Carol Birks, Matthew Kiernan
OBJECTIVE: To capture the clinical patterns, timing of key milestones and survival of patients presenting with amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) within Australia. METHODS: Data were prospectively collected and were timed to normal clinical assessments. An initial registration clinical report form (CRF) and subsequent ongoing assessment CRFs were submitted with a completion CRF at the time of death. DESIGN: Prospective observational cohort study...
September 30, 2016: BMJ Open
https://www.readbyqxmd.com/read/27679482/exosome-secretion-is-a-key-pathway-for-clearance-of-pathological-tdp-43
#20
Yohei Iguchi, Lara Eid, Martin Parent, Geneviève Soucy, Christine Bareil, Yuichi Riku, Kaori Kawai, Shinnosuke Takagi, Mari Yoshida, Masahisa Katsuno, Gen Sobue, Jean-Pierre Julien
Cytoplasmic TDP-43 aggregation is a pathological hallmark of amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Here we investigated the role of exosomes in the secretion and propagation of TDP-43 aggregates. TDP-43 was detected in secreted exosomes from Neuro2a cells and primary neurons but not from astrocytes or microglia. Evidence is presented that protein aggregation and autophagy inhibition are factors that promote exosomal secretion of TDP-43. We also report that levels of exosomal TDP-43 full length and C-terminal fragment species are upregulated in human amyotrophic lateral sclerosis brains...
December 2016: Brain: a Journal of Neurology
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