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https://www.readbyqxmd.com/read/29150766/spinobot-an-mri-guided-needle-positioning-system-for-spinal-cellular-therapeutics
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/28926131/region-specific-impairment-of-the-cervical-spinal-cord-sc-in-amyotrophic-lateral-sclerosis-a-preliminary-study-using-sc-templates-and-quantitative-mri-diffusion-tensor-imaging-inhomogeneous-magnetization-transfer
#12
Henitsoa Rasoanandrianina, Aude-Marie Grapperon, Manuel Taso, Olivier M Girard, Guillaume Duhamel, Maxime Guye, Jean-Philippe Ranjeva, Shahram Attarian, Annie Verschueren, Virginie Callot
In this preliminary study, our objective was to investigate the potential of high-resolution anatomical imaging, diffusion tensor imaging (DTI) and conventional/inhomogeneous magnetization transfer imaging [magnetization transfer (MT)/inhomogeneous magnetization transfer (ihMT)] at 3 T, analyzed with template-extracted regions of interest, to measure the atrophy and structural changes of white (WM) and gray (GM) matter spinal cord (SC) occurring in patients with amyotrophic lateral sclerosis (ALS). Ten patients with ALS and 20 age-matched healthy controls were recruited...
September 19, 2017: NMR in Biomedicine
https://www.readbyqxmd.com/read/28923025/intrafamilial-phenotypic-heterogeneity-in-a-taiwanese-family-with-a-mapt-p-r5h-mutation-a-case-report-and-literature-review
#13
Hui-Chi Lin, Chin-Hsien Lin, Pei-Lung Chen, Shih-Jung Cheng, Pei-Hao Chen
BACKGROUND: Frontotemporal degeneration (FTD) is a clinically and genetically heterogeneous neurodegenerative disorder characterized by deficits in executive function that frequently overlaps with parkinsonism and motor neuron disorders. Several genes have been identified to cause autosomal dominant forms of FTD, including the gene coding for the protein associated with microtubule tau (MAPT). While most reported pathogenic mutations in MAPT occur in exons 9-13, few families have been reported with mutations outside of this region...
September 18, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28891727/methodology-of-the-zoc-bhvi-high-myopia-cohort-study-the-onset-and-progression-of-myopic-pathologies-and-associated-risk-factors-in-highly-myopic-chinese
#14
Yanxian Chen, Ou Xiao, Xinxing Guo, Decai Wang, Padmaja Sankaridurg, Ian Morgan, Mingguang He
PURPOSE: The increasing prevalence of high myopia and its associated pathologies has raised challenges to ophthalmic services. This project aims to explore the onset and progression of myopic pathologies in highly myopic eyes through a prospective research cohort established in South China. METHODS: Patients with high myopia (sphere ≤ -6.00 D) visiting the optometric clinic of Zhongshan Ophthalmic Center (ZOC) were invited to participate in the baseline examinations and follow-up visit over a 10-year period...
September 11, 2017: Ophthalmic Epidemiology
https://www.readbyqxmd.com/read/28879883/the-role-of-diffusion-tensor-imaging-in-spinal-pathology-a-review
#15
REVIEW
Dan C Li, James G Malcolm, Rima S Rindler, Griffin R Baum, Avinash Rao, Shekar N Khurpad, Faiz U Ahmad
Diffusion tensor imaging (DTI) allows for noninvasive, in vivo visualization of white matter fiber tracts in the central nervous system by measuring the diffusion of water molecules. It provides both quantitative and qualitative (i.e., tractography) means to describe a region-of-interest. While protocols for the use of DTI are better established in the brain, the efficacy and potential applications of DTI in spinal cord pathology are less understood. In this review, we examine the current literature regarding the use of DTI in the spinal cord pathology, and in particular its diagnostic and prognostic value in traumatic injury, spinal tumors, cervical myelopathies, amyotrophic lateral sclerosis, and multiple sclerosis...
September 2017: Neurology India
https://www.readbyqxmd.com/read/28872040/structural-magnetic-resonance-imaging-in-frontotemporal-lobar-dementia
#16
Anne Bertrand, Sebastian Stroër, Isabelle Le Ber, Marc Teichmann, Didier Dormont
Frontotemporal lobar dementia (FTLD) is a heterogeneous group of neurodegenerative diseases. FTLD encompass: 1) behavioral forms, sometimes associated with amyotrophic lateral sclerosis; 2) linguistic forms (semantic and non-fluent primary progressive aphasia); 3) atypical parkinsonian syndromes (progressive supranuclear palsy and corticobasal syndrome). Standard brain MRI allows for strengthening the clinical suspicion of FTLD, by showing a pattern of atrophy in relation with the patient's clinical symptoms: frontotemporal anterior atrophy in behavioral forms; temporopolar or inferior left frontal atrophy in the linguistic forms; mesencephalic or corticosubcortical hemispheric atrophy in forms with atypical pakinsonism...
September 1, 2017: Gériatrie et Psychologie Neuropsychiatrie du Vieillissement
https://www.readbyqxmd.com/read/28855404/cognition-and-gray-and-white-matter-characteristics-of-presymptomatic-c9orf72-repeat-expansion
#17
Janne M Papma, Lize C Jiskoot, Jessica L Panman, Elise G Dopper, Tom den Heijer, Laura Donker Kaat, Yolande A L Pijnenburg, Lieke H Meeter, Rick van Minkelen, Serge A R B Rombouts, John C van Swieten
OBJECTIVE: To investigate cognitive function, gray matter volume, and white matter integrity in the presymptomatic stage of chromosome 9 open reading frame 72 repeat expansion (C9orf72RE). METHODS: Presymptomatic C9orf72RE carriers (n = 18) and first-degree family members without a pathogenic expansion (healthy controls [HC], n = 15) underwent a standardized protocol of neuropsychological tests, T1-weighted MRI, and diffusion tensor imaging within our cohort study of autosomal dominant frontotemporal dementia (FTD)...
September 19, 2017: Neurology
https://www.readbyqxmd.com/read/28828346/hirayama-s-disease-a-rare-clinical-variant-of-amyotrophic-lateral-sclerosis
#18
Swati C Aundhakar, Sanket K Mahajan, Daanish A Chhapra
Hirayama's disease is a rare clinical variant of amyotrophic lateral sclerosis where distal muscles are involved more compared to proximal muscles and vice-versa occurs only in 10% cases and so it is differentiated from O'Sullivan McLeod syndrome which involves only small muscles of single limb. Here, we present a case of Hirayama's disease where disease achieved a plateau after 3 years with no further progression. His electrophysiological studies, and clinical picture, and magnetic resonance imaging findings were consistent with a diagnosis of Hirayama's disease...
2017: Advanced Biomedical Research
https://www.readbyqxmd.com/read/28802959/a-combined-tract-based-spatial-statistics-and-voxel-based-morphometry-study-of-the-first-mri-scan-after-diagnosis-of-amyotrophic-lateral-sclerosis-with-subgroup-analysis
#19
A R Alruwaili, K Pannek, A Coulthard, R Henderson, N D Kurniawan, P McCombe
BACKGROUND AND PURPOSE: This study aims to compare the cortical and subcortical deep gray matter (GM) and white matter (WM) of ALS subjects and controls and to compare ALS subjects with (ALScog) and without (ALSnon-cog) cognitive impairment. MATERIALS AND METHODS: The study was performed in 30 ALS subjects, and 19 healthy controls. Structural T1- and diffusion-weighted MRI data were analyzed using voxel-based morphometry (VBM) and tract-based spatial statistics (TBSS)...
August 9, 2017: Journal of Neuroradiology. Journal de Neuroradiologie
https://www.readbyqxmd.com/read/28794983/multimodal-structural-mri-in-the-diagnosis-of-motor-neuron-diseases
#20
Pilar M Ferraro, Federica Agosta, Nilo Riva, Massimiliano Copetti, Edoardo Gioele Spinelli, Yuri Falzone, Gianni Sorarù, Giancarlo Comi, Adriano Chiò, Massimo Filippi
This prospective study developed an MRI-based method for identification of individual motor neuron disease (MND) patients and test its accuracy at the individual patient level in an independent sample compared with mimic disorders. 123 patients with amyotrophic lateral sclerosis (ALS), 44 patients with predominantly upper motor neuron disease (PUMN), 20 patients with ALS-mimic disorders, and 78 healthy controls were studied. The diagnostic accuracy of precentral cortical thickness and diffusion tensor (DT) MRI metrics of corticospinal and motor callosal tracts were assessed in a training cohort and externally proved in a validation cohort using a random forest analysis...
2017: NeuroImage: Clinical
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