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https://www.readbyqxmd.com/read/29321969/the-two-year-progression-of-structural-and-functional-cerebral-mri-in-amyotrophic-lateral-sclerosis
#1
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
#2
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/29287521/could-conservative-iron-chelation-lead-to-neuroprotection-in-amyotrophic-lateral-sclerosis
#3
Caroline Moreau, Véronique Danel, Jean Christophe Devedjian, Guillaume Grolez, Kelly Timmerman, Charlotte Laloux, Maud Petrault, Flore Gouel, Aurélie Jonneaux, Mary Dutheil, Cedrick Lachaud, Renaud Lopes, Gregory Kuchinski, Florent Auger, Maeva Kyheng, Alain Duhamel, Thierry Perez, Pierre-Francois Pradat, Helene Blasco, Charlotte Veyrat-Durebex, Philippe Corcia, Patrick Oeckl, Markus Otto, Luc Dupuis, Guillaume Garçon, Luc Defebvre, Ioav Zvi Cabantchik, James Duce, Régis Bordet, David Devos
Iron accumulation has been observed in mouse models and both sporadic and familial forms of Amyotrophic lateral sclerosis. 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 as compared with placebo...
December 29, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/29259550/longitudinal-diffusion-tensor-imaging-based-assessment-of-tract-alterations-an-application-to-amyotrophic-lateral-sclerosis
#4
Dobri Baldaranov, Andrei Khomenko, Ines Kobor, Ulrich Bogdahn, Martin Gorges, Jan Kassubek, Hans-Peter Müller
Objective: The potential of magnetic resonance imaging (MRI) as a technical biomarker for cerebral microstructural alterations in neurodegenerative diseases is under investigation. In this study, a framework for the longitudinal analysis of diffusion tensor imaging (DTI)-based mapping was applied to the assessment of predefined white matter tracts in amyotrophic lateral sclerosis (ALS), as an example for a rapid progressive neurodegenerative disease. Methods: DTI was performed every 3 months in six patients with ALS (mean (M) = 7...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/29250836/mr-scanning-tattoo-inks-and-risk-of-thermal-burn-an-experimental-study-of-iron-oxide-and-organic-pigments-effect-on-temperature-and-magnetic-behavior-referenced-to-chemical-analysis
#5
K K Alsing, H H Johannesen, R Hvass Hansen, M Dirks, O Olsen, J Serup
BACKGROUND: Tattooed persons examined with magnetic resonance imaging (MRI) can develop burning sensation suggested in the literature to be thermal burn from the procedure. MRI-induced thermal effect and magnetic behavior of known tattoo pigments were examined ex vivo. MATERIALS AND METHODS: Magnetic resonance imaging effects on 3 commonly used commercial ink stock products marketed for cosmetic tattooing was studied. A main study tested 22 formulations based on 11 pigment raw materials, for example, one line of 11 called pastes and another called dispersions...
December 17, 2017: Skin Research and Technology
https://www.readbyqxmd.com/read/29214883/longitudinal-structural-changes-in-als-a-three-time-point-imaging-study-of-white-and-gray-matter-degeneration
#6
Peter Bede, Orla Hardiman
BACKGROUND: Cross-sectional imaging studies offer valuable pathological insights into the neurodegenerative changes of amyotrophic lateral sclerosis. However, clinical trials urgently require sensitive monitoring markers that can detect subtle progressive changes over relatively short periods of time. We have conducted a three time-point longitudinal study to explore anatomical patterns of disease spread and to determine whether MRI metrics capture longitudinal changes over four and eight-month intervals...
December 7, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29197216/early-cognitive-structural-and-microstructural-changes-in-presymptomatic-c9orf72-carriers-younger-than-40-years
#7
Anne Bertrand, Junhao Wen, Daisy Rinaldi, Marion Houot, Sabrina Sayah, Agnès Camuzat, Clémence Fournier, Sabrina Fontanella, Alexandre Routier, Philippe Couratier, Florence Pasquier, Marie-Odile Habert, Didier Hannequin, Olivier Martinaud, Paola Caroppo, Richard Levy, Bruno Dubois, Alexis Brice, Stanley Durrleman, Olivier Colliot, Isabelle Le Ber
Importance: Presymptomatic carriers of chromosome 9 open reading frame 72 (C9orf72) mutation, the most frequent genetic cause of frontotemporal lobar degeneration and amyotrophic lateral sclerosis, represent the optimal target population for the development of disease-modifying drugs. Preclinical biomarkers are needed to monitor the effect of therapeutic interventions in this population. Objectives: To assess the occurrence of cognitive, structural, and microstructural changes in presymptomatic C9orf72 carriers...
December 2, 2017: JAMA Neurology
https://www.readbyqxmd.com/read/29175489/diffusion-tensor-imaging-identifies-presymptomatic-axonal-degeneration-in-the-spinal-cord-of-als-mice
#8
Rodolfo G Gatto, Weiguo Li, Richard L Magin
Extensive pathological evidence indicates that axonal degeneration represents an early and critical event in amyotrophic lateral sclerosis (ALS). Unfortunately, few MRI studies have focused in the early detection of white matter (WM) alterations in the spinal cord region. To unveil these WM changes, we performed high resolution diffusion tensor imaging (DTI) and correlated the results with histological analysis of adjacent slices taken from the spinal cords of presymptomatic mice. The DTI studies demonstrated a significant reduction in fractional anisotropy (FA) as well as axial diffusivities (AD) and an increase in radial diffusivity (RD), predominantly at lower segments of the spinal cord...
November 23, 2017: Brain Research
https://www.readbyqxmd.com/read/29175194/brain-signature-of-mild-stages-of-cognitive-and-behavioral-impairment-in-amyotrophic-lateral-sclerosis
#9
Lucas M T Branco, Thiago J R de Rezende, Caroline de O Roversi, Tamires Zanao, Raphael F Casseb, Brunno M de Campos, Marcondes C França
We aimed to assess the brain signature of cognitive and behavioral impairment in C9orf72-negative non-demented ALS patients. The study included 50 amyotrophic lateral sclerosis (ALS) patients (out of 75 initially recruited) and 38 healthy controls. High-resolution T1-weighted and spin-echo diffusion tensor images were acquired in a 3T MRI scanner. The multi atlas-based analysis protocol and the FreeSurfer tool were employed for gray matter assessment, and fiber tractography for white matter evaluation. Cognitively impaired ALS patients (n = 12) had bilateral amygdalae and left thalamic volumetric reduction compared to non-impaired ALS patients...
November 21, 2017: Psychiatry Research
https://www.readbyqxmd.com/read/29150766/spinobot-an-mri-guided-needle-positioning-system-for-spinal-cellular-therapeutics
#10
Alexander Squires, John N Oshinski, Nicholas M Boulis, Zion Tsz Ho Tse
The neurodegenerative disease amyotrophic lateral sclerosis (ALS) results in the death of motor neurons in voluntary muscles. There are no cures for ALS and few available treatments. In studies with small animal models, injection of cellular therapeutics into the anterior horn of the spinal cord has been shown to inhibit the progression of ALS. It was hypothesized that spinal injection could be made faster and less invasive with the aid of a robot. The robotic system presented-SpinoBot-uses MRI guidance to position a needle for percutaneous injection into the spinal cord...
November 17, 2017: Annals of Biomedical Engineering
https://www.readbyqxmd.com/read/29136131/characteristic-increases-in-eeg-connectivity-correlate-with-changes-of-structural-mri-in-amyotrophic-lateral-sclerosis
#11
Bahman Nasseroleslami, Stefan Dukic, Michael Broderick, Kieran Mohr, Christina Schuster, Brighid Gavin, Russell McLaughlin, Mark Heverin, Alice Vajda, Parameswaran M Iyer, Niall Pender, Peter Bede, Edmund C Lalor, Orla Hardiman
Amyotrophic lateral sclerosis (ALS) is a terminal progressive adult-onset neurodegeneration of the motor system. Although originally considered a pure motor degeneration, there is increasing evidence of disease heterogeneity with varying degrees of extra-motor involvement. How the combined motor and nonmotor degeneration occurs in the context of broader disruption in neural communication across brain networks has not been well characterized. Here, we have performed high-density crossectional and longitudinal resting-state electroencephalography (EEG) recordings on 100 ALS patients and 34 matched controls, and have identified characteristic patterns of altered EEG connectivity that have persisted in longitudinal analyses...
November 9, 2017: Cerebral Cortex
https://www.readbyqxmd.com/read/29131982/multimodal-mri-quantification-of-the-common-neurostructural-bases-within-the-ftd-als-continuum
#12
Chiara Crespi, Alessandra Dodich, Stefano F Cappa, Nicola Canessa, Sandro Iannaccone, Massimo Corbo, Christian Lunetta, Andrea Falini, Chiara Cerami
The continuum hypothesis linking the behavioral variant of frontotemporal dementia (bvFTD) and amyotrophic lateral sclerosis (ALS) is supported by clinical, pathological, genetic, and neuroimaging evidence. In the present multimodal magnetic resonance study, we characterized the site and extent of shared neurostructural changes in gray and white matter in 20 bvFTD and 19 ALS patients without dementia. We found an overlap of macrostructural and microstructural damage in both patient groups compared with healthy controls, involving the right orbital and the bilateral anterior cingulate cortices, the corticospinal tract and corpus callosum...
September 28, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29076800/neurodegeneration-of-brain-networks-in-the-amyotrophic-lateral-sclerosis-frontotemporal-lobar-degeneration-als-ftld-continuum-evidence-from-mri-and-meg-studies
#13
Francesca Trojsi, Pierpaolo Sorrentino, Giuseppe Sorrentino, Gioacchino Tedeschi
Brain imaging techniques, especially those based on magnetic resonance imaging (MRI) and magnetoencephalography (MEG), have been increasingly applied to study multiple large-scale distributed brain networks in healthy people and neurological patients. With regard to neurodegenerative disorders, amyotrophic lateral sclerosis (ALS), clinically characterized by the predominant loss of motor neurons and progressive weakness of voluntary muscles, and frontotemporal lobar degeneration (FTLD), the second most common early-onset dementia, have been proven to share several clinical, neuropathological, genetic, and neuroimaging features...
October 27, 2017: CNS Spectrums
https://www.readbyqxmd.com/read/29073458/resting-state-fmri-correlates-of-theory-of-mind-impairment-in-amyotrophic-lateral-sclerosis
#14
Francesca Trojsi, Federica Di Nardo, Gabriella Santangelo, Mattia Siciliano, Cinzia Femiano, Carla Passaniti, Giuseppina Caiazzo, Michele Fratello, Mario Cirillo, Maria Rosaria Monsurrò, Fabrizio Esposito, Gioacchino Tedeschi
Theory of Mind (ToM), the ability to recognize thoughts and emotions of another, may be one of the cognitive domains affected in amyotrophic lateral sclerosis (ALS), a neurodegenerative disease now recognized as a multi-system disorder. The present study aimed to identify early dysfunctions of brain resting state functional magnetic resonance imaging (RS-fMRI) networks in a group of ALS patients longitudinally explored for impairment of "cognitive" and "affective" ToM subcomponents. RS-fMRI connectivity was investigated in a group of 21 patients with ALS (i...
October 3, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/29071208/fast-progressive-lower-motor-neuron-disease-is-an-als-variant-a-two-centre-tract-of-interest-based-mri-data-analysis
#15
Hans-Peter Müller, Federica Agosta, Nilo Riva, Edoardo G Spinelli, Giancarlo Comi, Albert C Ludolph, Massimo Filippi, Jan Kassubek
BACKGROUND: The criteria for assessing upper motor neuron pathology in pure lower motor neuron disease (LMND) still remain a major issue of debate with respect to the clinical classification as an amyotrophic lateral sclerosis (ALS) variant. OBJECTIVE: The study was designed to investigate white matter damage by a hypothesis-guided tract-of-interest-based approach in patients with LMND compared with healthy controls and ´classical´ ALS patients in order to identify in vivo brain structural changes according to the neuropathologically defined ALS affectation pattern...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29055436/brain-white-matter-demyelinating-lesions-and-amyotrophic-lateral-sclerosis-in-a-patient-with-c9orf72-hexanucleotide-repeat-expansion
#16
Miguel Oliveira Santos, Inês Caldeira, Marta Gromicho, Ana Pronto-Laborinho, Mamede de Carvalho
A hexanucleotide repeat expansion in the C9orf72 gene is associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. It has been described before four patients with multiple sclerosis (MS) and C9orf72-ALS. However, C9orf72 positivity is not associated with increased risk of MS. Inflammatory pathways related to NF-κB have been linked to ALS and MS, and appear to be important in C9orf72-ALS patients. A 42-year-old woman presented with progressive bulbar symptoms for 9 months. Neurological examination disclosed spastic dysarthria, atrophic tongue with fasciculations, brisk jaw and limb tendon reflexes, and bilateral Hoffman sign...
October 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29021775/cardiac-findings-in-amyotrophic-lateral-sclerosis-a-magnetic-resonance-imaging-study
#17
Angela Rosenbohm, Benjamin Schmid, Dominik Buckert, Wolfgang Rottbauer, Jan Kassubek, Albert C Ludolph, Peter Bernhardt
The objective of this study was to investigate the potential involvement of cardiac structure and function by cardiac magnetic resonance (CMR) imaging in amyotrophic lateral sclerosis (ALS) patients. Our study included 35 patients with ALS without a history of cardiac disease and an age- and gender-matched healthy control group (n = 34). All subjects received a CMR in a 1.5-T whole-body scanner. Patients were also screened with Holter monitoring, echocardiography, and a blood test of cardiac markers. Myocardial mass in ALS hearts was reduced compared to the control group, and ejection volumes in the left and right heart were severely decreased in ALS patients, as shown by echocardiography and CMR...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28988390/topographic-distribution-of-brain-iron-deposition-and-small-cerebrovascular-lesions-in-amyotrophic-lateral-sclerosis-and-in-frontotemporal-lobar-degeneration-a-post-mortem-7-0-tesla-magnetic-resonance-imaging-study-with-neuropathological-correlates
#18
Jacques De Reuck, David Devos, Caroline Moreau, Florent Auger, Nicolas Durieux, Vincent Deramecourt, Florence Pasquier, Claude-Alain Maurage, Charlotte Cordonnier, Didier Leys, Regis Bordet
Amyotrophic lateral sclerosis (ALS) is associated with frontotemporal lobar degeneration (FTLD) in 15% of the cases. A neuropathological continuity between ALS and FTLD-TDP is suspected. The present post-mortem 7.0-tesla magnetic resonance imaging (MRI) study compares the topographic distribution of iron (Fe) deposition and the incidence of small cerebrovascular lesions in ALS and in FTLD brains. Seventy-eight post-mortem brains underwent 7.0-tesla MRI. The patients consisted of 12 with ALS, 38 with FTLD, and 28 controls...
October 7, 2017: Acta Neurologica Belgica
https://www.readbyqxmd.com/read/28952628/metal-complexes-for-multimodal-imaging-of-misfolded-protein-related-diseases
#19
S Lacerda, J-F Morfin, C F G C Geraldes, É Tóth
Aggregation of misfolded proteins and progressive polymerization of otherwise soluble proteins is a common hallmark of a wide range of highly debilitating and increasingly prevalent diseases, including amyotrophic lateral sclerosis, cerebral amyloid angiopathy, type II diabetes and Parkinson's, Huntington's and Alzheimer's diseases. There is a growing interest in creating imaging agents to detect such aggregates in various imaging modalities, including PET, SPECT and MRI. We present here an overview of recent efforts from the perspective of early diagnosis of amyloid diseases, with a major focus on Aβ detection and metal complexes bearing PiB units...
October 31, 2017: Dalton Transactions: An International Journal of Inorganic Chemistry
https://www.readbyqxmd.com/read/28926154/brain-functional-connectome-abnormalities-in-amyotrophic-lateral-sclerosis-are-associated-with-disability-and-cortical-hyperexcitability
#20
N Geevasinga, M S Korgaonkar, P Menon, M Van den Bos, L Gomes, S Foster, M C Kiernan, S Vucic
BACKGROUND AND PURPOSE: The present study utilized a multimodal approach encompassing connectome networks combined with brain volume analysis, and assessment of cortical excitability to provide novel insights into amyotrophic lateral sclerosis (ALS) pathogenesis. METHODS: Magnetic resonance images were acquired using a 3.0-Tesla Signa HDx scanner (GE Healthcare, Milwaukee, WI, USA), using an eight-channel head coil. Magnetic resonance images for the resting-state scan were acquired using an echo-planar imaging magnetic resonance sequence, acquiring 40 contiguous axial/oblique slices...
September 19, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
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