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https://www.readbyqxmd.com/read/28638712/frontal-assessment-battery-and-frontal-atrophy-in-amyotrophic-lateral-sclerosis
#1
Tatsuhiro Terada, Jun Miyata, Tomokazu Obi, Manabu Kubota, Miho Yoshizumi, Kinya Yamazaki, Kouichi Mizoguchi, Toshiya Murai
OBJECTIVES: To determine the potential utility of the frontal assessment battery (FAB) in assessing cognitive impairments in amyotrophic lateral sclerosis (ALS), we investigated the association between the FAB score and regional gray matter volume, and ascertained whether the regional brain alterations related to cognitive impairments occur in relatively mild stage of ALS. MATERIALS AND METHODS: Twenty-four ALS patients with a Mini-Mental State Examination score of >23, a normal score on the Self-Rating Depression Scale, little or no disturbance in speech and handling utensils on the ALS Functional Rating Scale (ALSFRS), and normal measures on respiratory tests (respiratory function test and arterial blood gas analysis), and two age-matched normal control groups (one for FAB assessment and the other for brain morphometry) underwent FAB testing and structural magnetic resonance imaging...
June 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28600740/functional-reorganization-during-cognitive-function-tasks-in-patients-with-amyotrophic-lateral-sclerosis
#2
Jürgen Keller, Sarah Böhm, Helena E A Aho-Özhan, Markus Loose, Martin Gorges, Jan Kassubek, Ingo Uttner, Sharon Abrahams, Albert C Ludolph, Dorothée Lulé
Cognitive deficits, especially in the domains of social cognition and executive function including verbal fluency, are common in amyotrophic lateral sclerosis (ALS) patients. There is yet sparse understanding of pathogenesis of the underlying, possibly adaptive, cortical patterns. To address this issue, 65 patients with ALS and 33 age-, gender- and education-matched healthy controls were tested on cognitive and behavioral deficits with the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). Using functional magnetic resonance imaging (fMRI), cortical activity during social cognition and executive function tasks (theory of mind, verbal fluency, alternation) adapted from the ECAS was determined in a 3 Tesla scanner...
June 9, 2017: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/28596251/hypothalamic-atrophy-is-related-to-body-mass-index-and-age-at-onset-in-amyotrophic-lateral-sclerosis
#3
Martin Gorges, Pauline Vercruysse, Hans-Peter Müller, Hans-Jürgen Huppertz, Angela Rosenbohm, Gabriele Nagel, Patrick Weydt, Åsa Petersén, Albert C Ludolph, Jan Kassubek, Luc Dupuis
OBJECTIVE: Our objective was to study the hypothalamic volume in a cohort of patients with amyotrophic lateral sclerosis (ALS) including symptomatic and presymptomatic ALS mutation carriers. METHODS: High-resolution three-dimensional T1-weighted MRI datasets from 251 patients with sporadic ALS, 19 symptomatic and 32 presymptomatic ALS mutation carriers and 112 healthy controls (HC) were retrospectivally registered for manual delineation of the hypothalamus. The volume of the hypothalamus, in total or subdivided, was normalised to the intracranial volume and adjusted to age...
June 8, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28591490/region-specific-atrophy-of-precentral-gyrus-in-patients-with-amyotrophic-lateral-sclerosis
#4
Yuanyuan Qin, Shun Zhang, Rifeng Jiang, Fei Gao, Xiaoying Tang, Wenzhen Zhu
PURPOSE: To assess the region-specific atrophy of precentral gyrus (PrCG) and its correlation to clinical function score in amyotrophic lateral sclerosis (ALS). MATERIALS AND METHODS: Twenty-eight patients with sporadic ALS and 28 healthy controls underwent high-resolution 3D T1-BRAVO magnetic resonance imaging at 3T. The bilateral PrCG segmentations were automatically obtained from a validated segmentation pipeline based on diffeomorphic multi-atlas likelihood fusion...
June 7, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/28591385/usefulness-of-diffusion-tensor-imaging-in-amyotrophic-lateral-sclerosis-potential-biomarker-and-association-with-the-cognitive-profile
#5
Marcelo Chaves, Mariela Bettini, Maria Cecilia Fernandez, Maria Jose Garcia Basalo, Juan Ignacio Rojas, Cristina Besada, Edgardo Cristiano, Angel Golimstok, Marcelo Rugiero
Methods: This was a case-control study conducted from December 1, 2012 to December 1, 2014. Clinical and demographic data were recorded. A neuropsychological test battery adapted to ALS patients was used. An MRI with DTI was performed in all patients and fractional anisotropy (FA) was analyzed in the white matter using the tract based spatial statistics program. Results: Twenty-four patients with ALS (15 females, mean age 66.9 + -2.3) and 13 healthy controls (four females, average age 66...
May 2017: Arquivos de Neuro-psiquiatria
https://www.readbyqxmd.com/read/28586096/spinal-cord-multi-parametric-magnetic-resonance-imaging-for-survival-prediction-in-amyotrophic-lateral-sclerosis
#6
G Querin, M M El Mendili, T Lenglet, S Delphine, V Marchand-Pauvert, H Benali, P-F Pradat
BACKGROUND AND PURPOSE: Assessing survival is a critical issue in patients with amyotrophic lateral sclerosis (ALS). Neuroimaging seems to be promising in the assessment of disease severity and several studies also suggest a strong relationship between spinal cord (SC) atrophy described by magnetic resonance imaging (MRI) and disease progression. The aim of the study was to determine the predictive added value of multimodal SC MRI on survival. METHODS: Forty-nine ALS patients were recruited and clinical data were collected...
June 6, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28575122/multimodal-assessment-of-white-matter-tracts-in-amyotrophic-lateral-sclerosis
#7
Florian Borsodi, Valeriu Culea, Christian Langkammer, Michael Khalil, Lukas Pirpamer, Stefan Quasthoff, Christian Enzinger, Reinhold Schmidt, Franz Fazekas, Stefan Ropele
Several quantitative magnetic resonance imaging (MRI) techniques have been proposed to investigate microstructural tissue changes in amyotrophic lateral sclerosis (ALS) including diffusion tensor imaging (DTI), magnetization transfer imaging, and R2* mapping. Here, in this study, we compared these techniques with regard to their capability for detecting ALS related white matter (WM) changes in the brain and their association with clinical findings. We examined 27 ALS patients and 35 age-matched healthy controls...
2017: PloS One
https://www.readbyqxmd.com/read/28572001/quantitative-flair-mri-in-amyotrophic-lateral-sclerosis
#8
Jeremy Fabes, Lucy Matthews, Nicola Filippini, Kevin Talbot, Mark Jenkinson, Martin R Turner
RATIONALE AND OBJECTIVES: T2-weighted magnetic resonance imaging (MRI) hyperintensity assessed visually in the corticospinal tract (CST) lacks sensitivity for a diagnosis of amyotrophic lateral sclerosis (ALS). We sought to explore a quantitative approach to fluid-attenuated inversion recovery (FLAIR) MRI intensity across a range of ALS phenotypes. MATERIALS AND METHODS: Thirty-three classical ALS patients, 10 with a flail arm presentation, and six with primary lateral sclerosis underwent MRI at 3 Tesla...
May 29, 2017: Academic Radiology
https://www.readbyqxmd.com/read/28544463/axonal-charcot-marie-tooth-neuropathy-concurrent-with-distal-and-proximal-weakness-by-translational-elongation-of-the-3-utr-in-nefh
#9
Da Eun Nam, Sung-Chul Jung, Da Hye Yoo, Sun Seong Choi, Sung-Yum Seo, Gwang Hoon Kim, Song-Ja Kim, Soo Hyun Nam, Byung-Ok Choi, Ki Wha Chung
Mutations in the NEFH gene encoding the heavy neurofilament protein are usually associated with neuronal damage and susceptibility to amyotrophic lateral sclerosis (ALS). Recently, frameshift variants in NEFH (p.Asp1004Glnfs*58 and p.Pro1008Alafs*56) have been reported to be the underlying cause of axonal Charcot-Marie-Tooth disease type 2CC (CMT2CC). The frameshift mutation resulted in a stop loss and translation of a cryptic amyloidogenic element (CAE) encoded by the 3' UTR. This study also identified a de novo c...
May 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28533943/etiology-and-treatment-of-amyotrophic-lateral-sclerosis
#10
Hernando Rafael, Juan Oscar David, Antonio Santiago Vilca
BACKGROUND: To date all researchers conclude that the etiology of Amyotrophic lateral sclerosis (ALS) is not known. On the contrary, since August 2009, we believe that disease is of ischemic origin in the anterior surface of the medulla oblongata. MATERIAL AND METHOD: We present our surgical experience into 45 patients with ALS (bulbar form in 36 cases and spinal form in 9). Preoperative MRI scans revealed microinfarcts in the medulla oblongata and/or cervical cord...
2017: American Journal of Neurodegenerative Disease
https://www.readbyqxmd.com/read/28529876/pathology-of-callosal-damage-in-als-an-ex-vivo-7%C3%A2-t-diffusion-tensor-mri-study
#11
Agustin M Cardenas, Joelle E Sarlls, Justin Y Kwan, Devin Bageac, Zachary S Gala, Laura E Danielian, Abhik Ray-Chaudhury, Hao-Wei Wang, Karla L Miller, Sean Foxley, Saad Jbabdi, Robert C Welsh, Mary Kay Floeter
OBJECTIVES: The goal of this study was to better understand the changes in tissue microstructure that underlie white matter diffusion changes in ALS patients. METHODS: Diffusion tensor imaging was carried out in postmortem brains of 4 ALS patients and two subjects without neurological disease on a 7 T MRI scanner using steady-state free precession sequences. Fractional anisotropy (FA) was measured in the genu, body, and splenium of the corpus callosum in formalin-fixed hemispheres...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28501822/structural-mri-correlates-of-amyotrophic-lateral-sclerosis-progression
#12
Joe Senda, Naoki Atsuta, Hirohisa Watanabe, Epifanio Bagarinao, Kazunori Imai, Daichi Yokoi, Yuichi Riku, Michihito Masuda, Ryoichi Nakamura, Hazuki Watanabe, Mizuki Ito, Masahisa Katsuno, Shinji Naganawa, Gen Sobue
PURPOSE: Amyotrophic lateral sclerosis (ALS) presents with varying degrees of brain degeneration that can extend beyond the corticospinal tract (CST). Furthermore, the clinical course and progression of ALS varies widely. Brain degeneration detected using structural MRI could reflect disease progression. SUBJECTS AND METHODS: On study registration, 3-Tesla volumetric MRI and diffusion tensor imaging scans were obtained at baseline in 38 healthy controls and 67 patients with sporadic ALS...
May 13, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28498852/ultra-high-field-7tesla-magnetic-resonance-spectroscopy-in-amyotrophic-lateral-sclerosis
#13
Nazem Atassi, Maosheng Xu, Christina Triantafyllou, Boris Keil, Robert Lawson, Paul Cernasov, Elena Ratti, Christopher J Long, Sabrina Paganoni, Alyssa Murphy, Nouha Salibi, Ravi Seethamraju, Bruce Rosen, Eva-Maria Ratai
The main objective of this study was to utilize high field (7T) in vivo proton magnetic resonance imaging to increase the ability to detect metabolite changes in people with ALS, specifically, to quantify levels of glutamine and glutamine separately. The second objective of this study was to correlate metabolic markers with clinical outcomes of disease progression. 13 ALS participants and 12 age-matched healthy controls (HC) underwent 7 Tesla MRI and MRS. Single voxel MR spectra were acquired from the left precentral gyrus using a very short echo time (TE = 5 ms) STEAM sequence...
2017: PloS One
https://www.readbyqxmd.com/read/28425061/gray-matter-and-white-matter-changes-in-non-demented-amyotrophic-lateral-sclerosis-patients-with-or-without-cognitive-impairment-a-combined-voxel-based-morphometry-and-tract-based-spatial-statistics-whole-brain-analysis
#14
Foteini Christidi, Efstratios Karavasilis, Franz Riederer, Ioannis Zalonis, Panagiotis Ferentinos, Georgios Velonakis, Sophia Xirou, Michalis Rentzos, Georgios Argiropoulos, Vasiliki Zouvelou, Thomas Zambelis, Athanasios Athanasakos, Panagiotis Toulas, Konstantinos Vadikolias, Efstathios Efstathopoulos, Spyros Kollias, Nikolaos Karandreas, Nikolaos Kelekis, Ioannis Evdokimidis
The phenotypic heterogeneity in amyotrophic lateral sclerosis (ALS) implies that patients show structural changes within but also beyond the motor cortex and corticospinal tract and furthermore outside the frontal lobes, even if frank dementia is not detected. The aim of the present study was to investigate both gray matter (GM) and white matter (WM) changes in non-demented amyotrophic lateral sclerosis (ALS) patients with or without cognitive impairment (ALS-motor and ALS-plus, respectively). Nineteen ALS-motor, 31 ALS-plus and 25 healthy controls (HC) underwent 3D-T1-weighted and 30-directional diffusion-weighted imaging on a 3 T MRI scanner...
April 19, 2017: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/28412941/survival-prediction-in-amyotrophic-lateral-sclerosis-based-on-mri-measures-and-clinical-characteristics
#15
Christina Schuster, Orla Hardiman, Peter Bede
BACKGROUND: Amyotrophic lateral sclerosis (ALS) a highly heterogeneous neurodegenerative condition. Accurate diagnostic, monitoring and prognostic biomarkers are urgently needed both for individualised patient care and clinical trials. A multimodal magnetic resonance imaging study is presented, where MRI measures of ALS-associated brain regions are utilised to predict 18-month survival. METHODS: A total of 60 ALS patients and 69 healthy controls were included in this study...
April 17, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28364332/repetitive-nerve-stimulation-as-a-diagnostic-aid-for-distinguishing-cervical-spondylotic-amyotrophy-from-amyotrophic-lateral-sclerosis
#16
Chaojun Zheng, Xiang Jin, Yu Zhu, Feizhou Lu, Jianyuan Jiang, Xinlei Xia
PURPOSE: To identify and compare the features of compound muscle action potential (CMAP) decrements in repetitive nerve stimulation (RNS) in patients with cervical spondylotic amyotrophy (CSA) and in patients with amyotrophic lateral sclerosis (ALS). METHODS: The cohort consisted of 43 CSA (distal-type to proximal-type ratio: 27-16) and 35 ALS patients. Five muscles, including abductor pollicis brevis (APB), abductor digiti minimi (ADM), biceps brachii (BB), middle deltoid (Del), and upper trapezius (Trap), were tested by 3-Hz RNS...
March 31, 2017: European Spine Journal
https://www.readbyqxmd.com/read/28351750/a-longitudinal-dti-and-histological-study-of-the-spinal-cord-reveals-early-pathological-alterations-in-g93a-sod1-mouse-model-of-amyotrophic-lateral-sclerosis
#17
Stefania Marcuzzo, Silvia Bonanno, Matteo Figini, Alessandro Scotti, Ileana Zucca, Ludovico Minati, Nilo Riva, Teuta Domi, Andrea Fossaghi, Angelo Quattrini, Barbara Galbardi, Sara D'Alessandro, Maria Grazia Bruzzone, José Manuel García-Verdugo, Victoria Moreno-Manzano, Renato Mantegazza, Pia Bernasconi
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective motor neuron degeneration in the motor cortex, brainstem and spinal cord. It is generally accepted that ALS is caused by death of motor neurons, however the exact temporal cascade of degenerative processes is not yet completely known. To identify the early pathological changes in spinal cord of G93A-SOD1 ALS mice we performed a comprehensive longitudinal analysis employing diffusion-tensor magnetic resonance imaging alongside histology and electron microscopy, in parallel with peripheral nerve histology...
March 27, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28337412/differential-involvement-of-corticospinal-tract-cst-fibers-in-umn-predominant-als-patients-with-or-without-cst-hyperintensity-a-diffusion-tensor-tractography-study
#18
Venkateswaran Rajagopalan, Erik P Pioro
Diagnosis of amyotrophic lateral sclerosis (ALS) depends on clinical evidence of combined upper motor neuron (UMN) and lower motor neuron (LMN) degeneration, although ALS patients can present with features predominantly of one or the other. Some UMN-predominant patients show hyperintense signal along the intracranial corticospinal tract (CST) on T2- and proton density (PD)-weighted images (ALS-CST +), and appear to have faster disease progression when compared to those without CST hyperintensity (ALS-CST -)...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28337409/network-degeneration-and-dysfunction-in-presymptomatic-c9orf72-expansion-carriers
#19
Suzee E Lee, Ana C Sias, Maria Luisa Mandelli, Jesse A Brown, Alainna B Brown, Anna M Khazenzon, Anna A Vidovszky, Theodore P Zanto, Anna M Karydas, Mochtar Pribadi, Deepika Dokuru, Giovanni Coppola, Dan H Geschwind, Rosa Rademakers, Maria Luisa Gorno-Tempini, Howard J Rosen, Bruce L Miller, William W Seeley
Hexanucleotide repeat expansions in C9ORF72 are the most common known genetic cause of familial and sporadic frontotemporal dementia and amyotrophic lateral sclerosis. Previous work has shown that patients with behavioral variant frontotemporal dementia due to C9ORF72 show salience and sensorimotor network disruptions comparable to those seen in sporadic behavioral variant frontotemporal dementia, but it remains unknown how early in the lifespan these and other changes in brain structure and function arise...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28274264/a-unique-pattern-of-cortical-connectivity-characterizes-patients-with-attention-deficit-disorders-a-large-electroencephalographic-coherence-study
#20
COMPARATIVE STUDY
Frank H Duffy, Aditi Shankardass, Gloria B McAnulty, Heidelise Als
BACKGROUND: Attentional disorders (ADD) feature decreased attention span, impulsivity, and over-activity interfering with successful lives. Childhood onset ADD frequently persists to adulthood. Etiology may be hereditary or disease associated. Prevalence is 5% but recognition may be 'overshadowed' by comorbidities (brain injury, mood disorder) thereby escaping formal recognition. Blinded diagnosis by MRI has failed. ADD may not itself manifest a single anatomical pattern of brain abnormality but may reflect multiple, unique responses to numerous and diverse etiologies...
March 9, 2017: BMC Medicine
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