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https://www.readbyqxmd.com/read/29751289/perfusion-alterations-converge-with-patterns-of-pathological-spread-in-transactive-response-dna-binding-protein-43-proteinopathies
#1
Pilar M Ferraro, Charles Jester, Christopher A Olm, Katerina Placek, Federica Agosta, Lauren Elman, Leo McCluskey, David J Irwin, John A Detre, Massimo Filippi, Murray Grossman, Corey T McMillan
Amyotrophic lateral sclerosis (ALS) and the behavioral variant of frontotemporal dementia (bvFTD) commonly share the presence of transactive response DNA-binding protein 43 (TDP-43) inclusions. Structural magnetic resonance imaging studies demonstrated evidence for TDP-43 pathology spread, but while structural imaging usually reveals overt neuronal loss, perfusion imaging may detect more subtle neural activity alterations. We evaluated perfusion as an early marker for incipient pathology-associated brain alterations in TDP-43 proteinopathies...
April 17, 2018: Neurobiology of Aging
https://www.readbyqxmd.com/read/29740862/integrated-mri-and-11-c-pbr28-pet-imaging-in-amyotrophic-lateral-sclerosis
#2
Mohamad J Alshikho, Nicole R Zürcher, Marco L Loggia, Paul Cernasov, Beverly Reynolds, Olivia Pijanowski, Daniel B Chonde, David Izquierdo Garcia, Caterina Mainero, Ciprian Catana, James Chan, Suma Babu, Sabrina Paganoni, Jacob M Hooker, Nazem Atassi
OBJECTIVE: To characterize [11 C]-PBR28 brain uptake using positron emission tomography (PET) in people with amyotrophic lateral sclerosis (ALS), and primary lateral sclerosis (PLS). We have previously shown increased [11 C]-PBR28 uptake in the precentral gyrus in a small group of ALS patients. Herein, we confirm our initial finding, study the longitudinal changes, and characterize the gray vs. white matter distribution of [11 C]-PBR28 uptake in a larger cohort of patients with ALS and PLS...
May 8, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/29716267/magnetic-resonance-imaging-based-anatomical-assessment-of-tongue-impairment-due-to-amyotrophic-lateral-sclerosis-a-preliminary-study
#3
Euna Lee, Fangxu Xing, Sung Ahn, Timothy G Reese, Ruopeng Wang, Jordan R Green, Nazem Atassi, Van J Wedeen, Georges El Fakhri, Jonghye Woo
Amyotrophic Lateral Sclerosis (ALS) is a neurological disorder, which impairs tongue function for speech and swallowing. A widely used Diffusion Tensor Imaging (DTI) analysis pipeline is employed for quantifying differences in tongue fiber myoarchitecture between controls and ALS patients. This pipeline uses both high-resolution magnetic resonance imaging (hMRI) and DTI. hMRI is used to delineate tongue muscles, while DTI provides indices to reveal fiber connectivity within and between muscles. The preliminary results using five controls and two patients show quantitative differences between the groups...
April 2018: Journal of the Acoustical Society of America
https://www.readbyqxmd.com/read/29706684/strain-map-of-the-tongue-in-normal-and-als-speech-patterns-from-tagged-and-diffusion-mri
#4
Fangxu Xing, Jerry L Prince, Maureen Stone, Timothy G Reese, Nazem Atassi, Van J Wedeen, Georges El Fakhri, Jonghye Woo
Amyotrophic Lateral Sclerosis (ALS) is a neurological disease that causes death of neurons controlling muscle movements. Loss of speech and swallowing functions is a major impact due to degeneration of the tongue muscles. In speech studies using magnetic resonance (MR) techniques, diffusion tensor imaging (DTI) is used to capture internal tongue muscle fiber structures in three-dimensions (3D) in a non-invasive manner. Tagged magnetic resonance images (tMRI) are used to record tongue motion during speech. In this work, we aim to combine information obtained with both MR imaging techniques to compare the functionality characteristics of the tongue between normal and ALS subjects...
February 2018: Proceedings of SPIE
https://www.readbyqxmd.com/read/29679161/present-and-future-of-ultra-high-field-mri-in-neurodegenerative-disorders
#5
REVIEW
Graziella Donatelli, Roberto Ceravolo, Daniela Frosini, Michela Tosetti, Ubaldo Bonuccelli, Mirco Cosottini
PURPOSE OF REVIEW: With a high signal-to-noise ratio, unparalleled spatial resolution, and improved contrasts, ultra-high field MR (≥ 7 T) has great potential in depicting the normal radiological anatomy of smaller structures in the brain and can also provide more information about morphological, quantitative, and metabolic changes associated with a wide range of brain disorders. By focusing attention on specific brain regions believed to be associated with early pathological change, or by more closely inspecting recognized foci of brain pathology, ultra-high field MR can improve the accuracy and sensitivity of neuroimaging...
April 20, 2018: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/29678369/impaired-corticomuscular-and-interhemispheric-cortical-beta-oscillation-coupling-in-amyotrophic-lateral-sclerosis
#6
Malcolm Proudfoot, Freek van Ede, Andrew Quinn, Giles L Colclough, Joanne Wuu, Kevin Talbot, Michael Benatar, Mark W Woolrich, Anna C Nobre, Martin R Turner
OBJECTIVES: The neural activity of the primary motor cortex is variably synchronised with contralateral peripheral electromyographic signals, which is thought to facilitate long-range communication through the motor system. Such corticomuscular coherence (CMC) is typically observed in the beta-band (15-30 Hz) range during steady force production. We aimed to measure pathological alteration to CMC resulting from ALS. METHODS: CMC was appraised during a forearm grip task in 17 ALS patients contrasted against age-matched healthy controls...
April 6, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/29620511/gray-matter-volume-changes-over-the-whole-brain-in-the-bulbar-and-spinal-onset-amyotrophic-lateral-sclerosis-a-voxel-based-morphometry-study
#7
Zhi-Ye Chen, Meng-Qi Liu, Lin Ma
Objective To investigate cerebral structural signatures of the bulbar- and spinal-onset amyotrophic lateral sclerosis (ALS) using voxel-based morphometry on magnetic resonance imaging. Methods The MR structural images of the brain were obtained from 65 ALS patients (15 bulbar-onset, 50 spinal-onset) and 65 normal controls (NC) on a 3.0T MRI system. Gray matter (GM) volume changes were investigated by voxel-based morphometry, and the distribution of the brain regions with volume changes was compared between ALS and normal controls, as well as between bulbar-onset and spinal-onset ALS based on Neuromorphometrics atlas...
March 30, 2018: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/29599716/atrophy-in-the-thalamus-but-not-cerebellum-is-specific-for-c9orf72-ftd-and-als-patients-an-atlas-based-volumetric-mri-study
#8
Sonja Schönecker, Christiane Neuhofer, Markus Otto, Albert Ludolph, Jan Kassubek, Bernhard Landwehrmeyer, Sarah Anderl-Straub, Elisa Semler, Janine Diehl-Schmid, Catharina Prix, Christian Vollmar, Juan Fortea, Hans-Jürgen Huppertz, Thomas Arzberger, Dieter Edbauer, Berend Feddersen, Marianne Dieterich, Matthias L Schroeter, Alexander E Volk, Klaus Fließbach, Anja Schneider, Johannes Kornhuber, Manuel Maler, Johannes Prudlo, Holger Jahn, Tobias Boeckh-Behrens, Adrian Danek, Thomas Klopstock, Johannes Levin
Background: The neuropathology of patients with frontotemporal dementia (FTD) or amyotrophic lateral sclerosis (ALS) due to a C9orf72 mutation is characterized by two distinct types of characteristic protein depositions containing either TDP-43 or so-called dipeptide repeat proteins that extend beyond frontal and temporal regions. Thalamus and cerebellum seem to be preferentially affected by the dipeptide repeat pathology unique to C9orf72 mutation carriers. Objective: This study aimed to determine if mutation carriers showed an enhanced degree of thalamic and cerebellar atrophy compared to sporadic patients or healthy controls...
2018: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/29577934/selective-spatio-temporal-vulnerability-of-central-nervous-system-neurons-to-pathological-tar-dna-binding-protein-43-in-aged-transgenic-mice
#9
Annika van Hummel, Gabriella Chan, Julia van der Hoven, Marco Morsch, Stefania Ippati, Lisa Suh, Mian Bi, Prita R Asih, Wei Siang Lee, Troy A Butler, Magdalena Przybyla, Glenda M Halliday, Olivier Piguet, Matthew C Kiernan, Roger S Chung, Lars M Ittner, Yazi D Ke
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing and fatal disease characterized by muscular atrophy due to loss of upper and lower motor neurons. Histopathologically, the majority of ALS cases present with abnormal cytoplasmic accumulation and aggregation of the nuclear RNA-regulating protein TAR DNA-binding protein 43 (TDP-43). Pathogenic mutations in the TARDBP gene that encodes TDP-43 have been identified in familial ALS. We have previously reported transgenic mice with neuronal expression of human TDP-43 carrying the pathogenic A315T mutation (iTDP-43A315T mice), presenting with early-onset motor deficits in adolescent animals...
March 22, 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29564498/beyond-fractional-anisotropy-in-amyotrophic-lateral-sclerosis-the-value-of-mean-axial-and-radial-diffusivity-and-its-correlation-with-electrophysiological-conductivity-changes
#10
Ana Filipa Geraldo, João Pereira, Pedro Nunes, Sofia Reimão, Rita Sousa, Miguel Castelo-Branco, Susana Pinto, Jorge Guedes Campos, Mamede de Carvalho
PURPOSE: This paper aims to analyze the contribution of mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) in the detection of microstructural abnormalities in amyotrophic lateral sclerosis (ALS) and to evaluate the degree of agreement between structural and functional changes through concomitant diffusion tensor imaging (DTI), transcranial magnetic stimulation (TMS), and clinical assessment. METHODS: Fourteen patients with ALS and 11 healthy, age- and gender-matched controls were included...
May 2018: Neuroradiology
https://www.readbyqxmd.com/read/29529995/dissecting-the-pathobiology-of-altered-mri-signal-in-amyotrophic-lateral-sclerosis-a-post-mortem-whole-brain-sampling-strategy-for-the-integration-of-ultra-high-field-mri-and-quantitative-neuropathology
#11
Menuka Pallebage-Gamarallage, Sean Foxley, Ricarda A L Menke, Istvan N Huszar, Mark Jenkinson, Benjamin C Tendler, Chaoyue Wang, Saad Jbabdi, Martin R Turner, Karla L Miller, Olaf Ansorge
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a clinically and histopathologically heterogeneous neurodegenerative disorder, in which therapy is hindered by the rapid progression of disease and lack of biomarkers. Magnetic resonance imaging (MRI) has demonstrated its potential for detecting the pathological signature and tracking disease progression in ALS. However, the microstructural and molecular pathological substrate is poorly understood and generally defined histologically...
March 13, 2018: BMC Neuroscience
https://www.readbyqxmd.com/read/29477906/cognitive-phenotypes-of-sequential-staging-in-amyotrophic-lateral-sclerosis
#12
Dorothée Lulé, Sarah Böhm, Hans-Peter Müller, Helena Aho-Özhan, Jürgen Keller, Martin Gorges, Markus Loose, Jochen H Weishaupt, Ingo Uttner, Elmar Pinkhardt, Jan Kassubek, Kelly Del Tredici, Heiko Braak, Sharon Abrahams, Albert C Ludolph
Sequential spread of TDP-43 load in the brain may be a pathological characteristic of amyotrophic lateral sclerosis (ALS). Diffusion tensor imaging (DTI) is a magnetic resonance imaging (MRI) based marker of this pathological feature. Cognitive deficits known to be present in a subset of ALS patients might act as an additional in vivo clinical marker of disease spread. N = 139 patients with ALS were tested with the Edinburgh Cognitive and Behavioural ALS screen (ECAS) in addition to DTI brain measures of pathological spread...
April 2018: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29405031/imaging-techniques-in-als
#13
Fulvio Zaccagna, Giulia Lucignani, Eytan Raz, Claudio Colonnese
Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease characterized by degeneration of both upper and lower motor neuron located in the spinal cord and brainstem. Diagnosis of ALS is predominantly clinical, nevertheless, electromyography and Magnetic Resonance Imaging (MRI) may provide support. Several advanced MRI techniques have been proven useful for ALS diagnosis and, indeed, the combination of different MRI techniques demonstrated an improvement in sensitivity and specificity as far as 90%...
December 1, 2017: Archives Italiennes de Biologie
https://www.readbyqxmd.com/read/29379040/mri-of-the-cervical-spinal-cord-predicts-respiratory-dysfunction-in-als
#14
G Grolez, M Kyheng, R Lopes, C Moreau, K Timmerman, F Auger, G Kuchcinski, A Duhamel, P Jissendi-Tchofo, P Besson, C Laloux, M Petrault, J C Devedjian, Thierry Pérez, Pierre François Pradat, L Defebvre, R Bordet, V Danel-Brunaud, D Devos
For patients with amyotrophic lateral sclerosis (ALS), the primary therapeutic goal is to minimize morbidity. Non-invasive ventilation improves survival. We aim to assess whether Magnetic Resonance Imaging (MRI) of the cervical spinal cord predicts the progression of respiratory disorders in ALS. Brain and spinal MRI was repeatedly performed in the SOD1G86R mouse model, in 40 patients and in healthy controls. Atrophy, iron overload, white matter diffusivity and neuronal loss were assessed. In Superoxide Dismutase-1 (SOD1) mice, iron accumulation appeared in the cervical spinal cord at symptom onset but disappeared with disease progression (after the onset of atrophy)...
January 29, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29374846/structural-and-functional-papez-circuit-integrity-in-amyotrophic-lateral-sclerosis
#15
Ana Paula Arantes Bueno, Walter Hugo L Pinaya, Luciana M Moura, Maxime Bertoux, Ratko Radakovic, Matthew C Kiernan, Antonio Lucio Teixeira, Leonardo Cruz de Souza, Michael Hornberger, João Ricardo Sato
Cognitive impairment in amyotrophic lateral sclerosis (ALS) is heterogeneous but now recognized as a feature in non-demented patients and no longer exclusively attributed to executive dysfunction. However, despite common reports of temporal lobe changes and memory deficits in ALS, episodic memory has been less explored. In the current study, we examined how the Papez circuit-a circuit known to participate in memory processes-is structurally and functionally affected in ALS patients (n = 20) compared with healthy controls (n = 15), and whether these changes correlated with a commonly used clinical measure of episodic memory...
January 27, 2018: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/29367447/unraveling-als-due-to-sod1-mutation-through-the-combination-of-brain-and-cervical-cord-mri
#16
Federica Agosta, Edoardo Gioele Spinelli, Ivan V Marjanovic, Zorica Stevic, Elisabetta Pagani, Paola Valsasina, Biljana Salak-Djokic, Milena Jankovic, Dragana Lavrnic, Vladimir S Kostic, Massimo Filippi
OBJECTIVE: To explore structural and functional changes of the brain and cervical cord in patients with amyotrophic lateral sclerosis (ALS) due to mutation in the superoxide dismutase (SOD1) gene compared with sporadic ALS. METHODS: Twenty patients with SOD1 ALS, 11 with sporadic ALS, and 33 healthy controls underwent clinical evaluation and brain MRI. Cortical thickness analysis, diffusion tensor MRI of the corticospinal tracts (CST) and corpus callosum, and resting-state functional connectivity were performed...
January 24, 2018: Neurology
https://www.readbyqxmd.com/read/29356968/low-signal-intensity-in-motor-cortex-on-susceptibility-weighted-mr-imaging-is-correlated-with-clinical-signs-of-amyotrophic-lateral-sclerosis-a-pilot-study
#17
Hironobu Endo, Kenji Sekiguchi, Hitoshi Shimada, Takehiro Ueda, Hisatomo Kowa, Fumio Kanda, Tatsushi Toda
There is no reliable objective indicator for upper motor neuron dysfunction in amyotrophic lateral sclerosis (ALS). To determine the clinical significance and potential utility of magnetic resonance (MR) signals, we investigated the relationship between clinical symptoms and susceptibility changes in the motor cortex measured using susceptibility-weighted MR imaging taken by readily available 3-T MRI in clinical practice. Twenty-four ALS patients and 14 control subjects underwent 3-T MR T1-weighted imaging and susceptibility-weighted MR imaging with the principles of echo-shifting with a train of observations (PRESTO) sequence...
March 2018: Journal of Neurology
https://www.readbyqxmd.com/read/29321969/the-two-year-progression-of-structural-and-functional-cerebral-mri-in-amyotrophic-lateral-sclerosis
#18
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/29316850/demographics-and-clinical-characteristics-of-primary-lateral-sclerosis-case-series-and-a-review-of-literature
#19
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...
February 2018: Neurodegenerative Disease Management
https://www.readbyqxmd.com/read/29287521/could-conservative-iron-chelation-lead-to-neuroprotection-in-amyotrophic-lateral-sclerosis
#20
Caroline Moreau, Véronique Danel, Jean Christophe Devedjian, Guillaume Grolez, Kelly Timmerman, Charlotte Laloux, Maud Petrault, Flore Gouel, Aurélie Jonneaux, Mary Dutheil, Cédrick Lachaud, Renaud Lopes, Grégory Kuchcinski, Florent Auger, Maeva Kyheng, Alain Duhamel, Thierry Pérez, Pierre François Pradat, Hélène Blasco, Charlotte Veyrat-Durebex, Philippe Corcia, Patrick Oeckl, Markus Otto, Luc Dupuis, Guillaume Garçon, Luc Defebvre, Z Ioav Cabantchik, James Duce, Régis Bordet, David Devos
Iron accumulation has been observed in mouse models and in both sporadic and familial forms of amyotrophic lateral sclerosis (ALS). Iron chelation could reduce iron accumulation and the related excess of oxidative stress in the motor pathways. However, classical iron chelation would induce systemic iron depletion. We assess the safety and efficacy of conservative iron chelation (i.e., chelation with low risk of iron depletion) in a murine preclinical model and pilot clinical trial. In Sod1G86R mice, deferiprone increased the mean life span compared with placebo...
February 8, 2018: Antioxidants & Redox Signaling
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