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https://www.readbyqxmd.com/read/29748771/inhibition-impairment-in-frontotemporal-dementia-amyotrophic-lateral-sclerosis-and-alzheimer-s-disease-clinical-assessment-and-metabolic-correlates
#1
Jordi A Matías-Guiu, María Nieves Cabrera-Martín, María Valles-Salgado, Teresa Rognoni, Lucía Galán, Teresa Moreno-Ramos, José Luis Carreras, Jorge Matías-Guiu
The ability to reject an automatic tendency, i.e. inhibition, has been linked to the prefrontal cortex, but its neural underpinnings are still controversial. Neurodegenerative diseases represent an interesting model to explore this issue, given its frequent impairment in these disorders. We investigated the inhibitory impairment and its neural basis using four different tests, which evaluate the presence of inhibitory dysfunction (Stroop test, Hayling test, and two graphical perseveration tests), and assessed their correlation with brain metabolism using 18 F-fluorodeoxyglucose positron emission tomography in a group of 76 participants with behavioral variant frontotemporal dementia (bvFTD), Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS) and healthy controls (HC)...
May 10, 2018: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/29734264/the-accuracy-of-ineco-frontal-screening-in-the-diagnosis-of-executive-dysfunction-in-frontotemporal-dementia-and-alzheimer-disease
#2
Valéria S Bahia, Mário A Cecchini, Luciana Cassimiro, Rene Viana, Thais B Lima-Silva, Leonardo Cruz de Souza, Viviane Amaral Carvalho, Henrique C Guimarães, Paulo Caramelli, Márcio L F Balthazar, Benito Damasceno, Sônia M D Brucki, Ricardo Nitrini, Mônica S Yassuda
INTRODUCTION: Executive dysfunction is a common symptom in neurodegenerative disorders and is in need of easy-to-apply screening tools that might identify it. The aims of the present study were to examine some of the psychometric characteristics of the Brazilian version of the INECO frontal screening (IFS), and to investigate its accuracy to diagnose executive dysfunction in dementia and its accuracy to differentiate Alzheimer disease (AD) from the behavioral variant of frontotemporal dementia (bvFTD)...
May 4, 2018: Alzheimer Disease and Associated Disorders
https://www.readbyqxmd.com/read/29615896/brain-aging-and-apoe-%C3%AE%C2%B54-interact-to-reveal-potential-neuronal-compensation-in-healthy-older-adults
#3
Elisa Scheller, Lena V Schumacher, Jessica Peter, Jacob Lahr, Julius Wehrle, Christoph P Kaller, Christian Gaser, Stefan Klöppel
Compensation implies the recruitment of additional neuronal resources to prevent the detrimental effect of age-related neuronal decline on cognition. Recently suggested statistical models comprise behavioral performance, brain activation, and measures related to aging- or disease-specific pathological burden to characterize compensation. Higher chronological age as well as the APOE ε4 allele are risk factors for Alzheimer's disease. A more biological approach to characterize aging compared with chronological age is the brain age gap estimation (BrainAGE), taking into account structural brain characteristics...
2018: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/29614676/regional-distribution-asymmetry-and-clinical-correlates-of-tau-uptake-on-18f-av-1451-pet-in-atypical-alzheimer-s-disease
#4
Katerina A Tetzloff, Jonathan Graff-Radford, Peter R Martin, Nirubol Tosakulwong, Mary M Machulda, Joseph R Duffy, Heather M Clark, Matthew L Senjem, Christopher G Schwarz, Anthony J Spychalla, Daniel A Drubach, Clifford R Jack, Val J Lowe, Keith A Josephs, Jennifer L Whitwell
BACKGROUND: Despite common pathology, Alzheimer's disease (AD) can have multiple clinical presentations which pathological studies suggest result from differences in the regional distribution of tau pathology. Positron emission tomography (PET) ligands are now available that can detect tau proteins in vivo and hence can be used to investigate the biological mechanisms underlying atypical AD. OBJECTIVE: To assess regional patterns of tau uptake on PET imaging in two atypical AD variants, posterior cortical atrophy (PCA) and logopenic progressive aphasia (lvPPA)...
2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29614645/structural-anatomical-investigation-of-long-term-memory-deficit-in-behavioral-frontotemporal-dementia
#5
Maxime Bertoux, Emma C Flanagan, Matthew Hobbs, Amparo Ruiz-Tagle, Carolina Delgado, Marcelo Miranda, Agustín Ibáñez, Andrea Slachevsky, Michael Hornberger
Although a growing body of work has shown that behavioral variant frontotemporal dementia (bvFTD) could present with severe amnesia in approximately half of cases, memory assessment is currently the clinical standard to distinguish bvFTD from Alzheimer's disease (AD). Thus, the concept of "relatively preserved episodic memory" in bvFTD remains the basis of its clinical distinction from AD and a criterion for bvFTD's diagnosis. This view is supported by the idea that bvFTD is not characterized by genuine amnesia and hippocampal degeneration, by contrast to AD...
March 24, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29573041/apoe-influences-working-memory-in-non-demented-elderly-through-an-interaction-with-spon1-rs2618516
#6
Zhen Liu, Xiangwei Dai, Wuhai Tao, Huilan Liu, He Li, Caishui Yang, Junying Zhang, Xin Li, Yaojing Chen, Chao Ma, Jing Pei, Haohao Mao, Kewei Chen, Zhanjun Zhang
Exploring how risk genes cumulatively impair brain function in preclinical phase (i.e., in cognitively normal elderly) could provide critical insights into the pathophysiology of Alzheimer's disease (AD). Working memory impairment has always been a considerable cognitive deficit in AD, which is likely under complex genetic control. Though, the APOE ɛ4 allele could damage the working memory performance in normal elderly, dissociable results have been reported. This allele may exert specific effects in contexts with other genetic variants...
March 24, 2018: Human Brain Mapping
https://www.readbyqxmd.com/read/29569990/molecular-neuroimaging-in-primary-progressive-aphasia-with-predominant-agraphia
#7
Rene L Utianski, Joseph R Duffy, Rodolfo Savica, Jennifer L Whitwell, Mary M Machulda, Keith A Josephs
A 62-year-old male presented with progressive isolated writing and spelling difficulties. Neurological, neuropsychological, speech, and language evaluations identified only minimal additional abnormalities. The presenting characteristics did not meet criteria for any particular variant of primary progressive aphasia; his clinical presentation is best described as primary progressive aphasia, with a predominant, almost pure agraphia. Brain MRI showed asymmetric, bilateral parenchymal volume loss, with left hippocampal atrophy...
March 23, 2018: Neurocase
https://www.readbyqxmd.com/read/29554554/predominant-subcortical-accumulation-of-18-f-flortaucipir-binding-in-behavioral-variant-frontotemporal-dementia
#8
Hanna Cho, Sang Won Seo, Jae Yong Choi, Hye Sun Lee, Young Hoon Ryu, Myung Sik Lee, Duk L Na, Hee Jin Kim, Chul Hyoung Lyoo
Behavioral variant frontotemporal dementia (bvFTD) is the most common form of frontotemporal dementia, and tau pathology can be found in 40%-50% of bvFTD patients. In this study, we sought to investigate 18 F-flortaucipir-binding patterns and their correlates in clinically diagnosed bvFTD patients by comparing with results for Alzheimer's disease (AD) patients. We enrolled 20 bvFTD, 20 AD, and 20 age-matched healthy subjects who underwent neuropsychological tests, magnetic resonance imaging, and tau positron emission tomography scans with 18 F-flortaucipir...
June 2018: Neurobiology of Aging
https://www.readbyqxmd.com/read/29514157/cognitive-reserve-hypothesis-in-frontotemporal-dementia-evidence-from-a-brain-spect-study-in-a-series-of-greek-frontotemporal-dementia-patients
#9
Pantelis Maiovis, Panagiotis Ioannidis, Georgios Gerasimou, Anna Gotzamani-Psarrakou, Dimitrios Karacostas
BACKGROUND AND OBJECTIVE: Cognitive reserve (CR) mediates the clinical expression of brain pathology in Alzheimer's disease, while there are much less relevant data in frontotemporal dementia (FTD). In the present study we examined whether CR, measured using the Cognitive Reserve Index (CRI), correlated with regional cerebral blood flow (rCBF) in Greek FTD patients. METHODS: Eighty FTD patients, i.e., 47 with behavioral variant FTD (bvFTD) and 33 with primary progressive aphasia (PPA), were enrolled into this study...
March 7, 2018: Neuro-degenerative Diseases
https://www.readbyqxmd.com/read/29503034/alterations-in-cholesterol-metabolism-related-genes-in-sporadic-alzheimer-s-disease
#10
Cynthia Picard, Cédric Julien, Josée Frappier, Justin Miron, Louise Théroux, Doris Dea, John C S Breitner, Judes Poirier
Genome-wide association studies have identified several cholesterol metabolism-related genes as top risk factors for late-onset Alzheimer's disease (LOAD). We hypothesized that specific genetic variants could act as disease-modifying factors by altering the expression of those genes. Targeted association studies were conducted with available genomic, transcriptomic, proteomic, and histopathological data from 3 independent cohorts: the Alzheimer's Disease Neuroimaging Initiative (ADNI), the Quebec Founder Population (QFP), and the United Kingdom Brain Expression Consortium (UKBEC)...
June 2018: Neurobiology of Aging
https://www.readbyqxmd.com/read/29473050/-cyp2c19-variant-mitigates-alzheimer-disease-pathophysiology-in-vivo-and-postmortem
#11
Andréa L Benedet, Lei Yu, Aurélie Labbe, Sulantha Mathotaarachchi, Tharick A Pascoal, Monica Shin, Min-Su Kang, Serge Gauthier, Guy A Rouleau, Judes Poirier, David A Bennett, Pedro Rosa-Neto
Objective: To verify whether CYP polymorphisms are associated with amyloid-β (Aβ) pathology across the spectrum of clinical Alzheimer disease using in vivo and postmortem data from 2 independent cohorts. Methods: A candidate-gene approach tested the association between 5 genes (28 single nucleotide polymorphisms) and Aβ load measured in vivo by the global [18 F]florbetapir PET standardized uptake value ratio (SUVR) in 338 Alzheimer's Disease Neuroimaging Initiative participants...
February 2018: Neurology. Genetics
https://www.readbyqxmd.com/read/29403351/hemoglobin-mrna-changes-in-the-frontal-cortex-of-patients-with-neurodegenerative-diseases
#12
Silvia Vanni, Marco Zattoni, Fabio Moda, Giorgio Giaccone, Fabrizio Tagliavini, Stéphane Haïk, Jean-Philippe Deslys, Gianluigi Zanusso, James W Ironside, Margarita Carmona, Isidre Ferrer, Gabor G Kovacs, Giuseppe Legname
Background: Hemoglobin is the major protein found in erythrocytes, where it acts as an oxygen carrier molecule. In recent years, its expression has been reported also in neurons and glial cells, although its role in brain tissue remains still unknown. Altered hemoglobin expression has been associated with various neurodegenerative disorders. Here, we investigated hemoglobin mRNA levels in brains of patients affected by variant, iatrogenic, and sporadic forms of Creutzfeldt-Jakob disease (vCJD, iCJD, sCJD, respectively) and in different genetic forms of prion diseases (gPrD) in comparison to Alzheimer's disease (AD) subjects and age-matched controls...
2018: Frontiers in Neuroscience
https://www.readbyqxmd.com/read/29387532/fdg-pet-and-csf-biomarker-accuracy-in-prediction-of-conversion-to-different-dementias-in-a-large-multicentre-mci-cohort
#13
Silvia Paola Caminiti, Tommaso Ballarini, Arianna Sala, Chiara Cerami, Luca Presotto, Roberto Santangelo, Federico Fallanca, Emilia Giovanna Vanoli, Luigi Gianolli, Sandro Iannaccone, Giuseppe Magnani, Daniela Perani
Background/aims: In this multicentre study in clinical settings, we assessed the accuracy of optimized procedures for FDG-PET brain metabolism and CSF classifications in predicting or excluding the conversion to Alzheimer's disease (AD) dementia and non-AD dementias. Methods: We included 80 MCI subjects with neurological and neuropsychological assessments, FDG-PET scan and CSF measures at entry, all with clinical follow-up. FDG-PET data were analysed with a validated voxel-based SPM method...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29376863/altered-expression-of-circulating-cdc42-in-frontotemporal-lobar-degeneration
#14
Claudia Saraceno, Marcella Catania, Anna Paterlini, Silvia Fostinelli, Miriam Ciani, Roberta Zanardini, Giuliano Binetti, Giuseppe Di Fede, Paola Caroppo, Luisa Benussi, Roberta Ghidoni, Silvia Bolognin
The term frontotemporal lobar degeneration (FTLD) defines a group of heterogeneous conditions histologically characterized by neuronal degeneration, inclusions of various proteins, and synaptic loss. However, the molecular mechanisms contributing to these alterations are still unknown. As the Rho-GTPase family member Cell division cycle 42 (Cdc42) plays a key role in the regulation of actin cytoskeleton dynamics and spine formation, we investigated whether Cdc42 protein levels were altered in the disease. Cdc42 was increased in the frontal cortex of FTLD patients compared to age-matched controls, but also in Alzheimer's disease (AD) patients included in the data-set...
2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29259854/microglia-express-abi3-in-the-brains-of-alzheimer-s-disease-and-nasu-hakola-disease
#15
Jun-Ichi Satoh, Yoshihiro Kino, Motoaki Yanaizu, Youhei Tosaki, Kenji Sakai, Tsuyoshi Ishida, Yuko Saito
Nasu-Hakola disease (NHD) is a rare autosomal recessive leukoencephalopathy caused by a loss-of-function mutation of either TYROBP ( DAP12 ) or TREM2 expressed in microglia. A rare variant of the TREM2 gene encoding p.Arg47His causes a 3-fold increase in the risk for late-onset Alzheimer's disease (LOAD). A recent study demonstrated that a rare coding variant p.Ser209Phe in the ABI family member 3 ( ABI3 ) gene, a regulator of actin cytoskeleton organization, confers risk of developing of LOAD, although the pattern of ABI3 expression in AD and NHD brains with relevance to microglial pathology remains to be characterized...
November 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/29158413/amyloid-polymorphisms-constitute-distinct-clouds-of-conformational-variants-in-different-etiological-subtypes-of-alzheimer-s-disease
#16
Jay Rasmussen, Jasmin Mahler, Natalie Beschorner, Stephan A Kaeser, Lisa M Häsler, Frank Baumann, Sofie Nyström, Erik Portelius, Kaj Blennow, Tammaryn Lashley, Nick C Fox, Diego Sepulveda-Falla, Markus Glatzel, Adrian L Oblak, Bernardino Ghetti, K Peter R Nilsson, Per Hammarström, Matthias Staufenbiel, Lary C Walker, Mathias Jucker
The molecular architecture of amyloids formed in vivo can be interrogated using luminescent conjugated oligothiophenes (LCOs), a unique class of amyloid dyes. When bound to amyloid, LCOs yield fluorescence emission spectra that reflect the 3D structure of the protein aggregates. Given that synthetic amyloid-β peptide (Aβ) has been shown to adopt distinct structural conformations with different biological activities, we asked whether Aβ can assume structurally and functionally distinct conformations within the brain...
December 5, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29142239/differential-overexpression-of-serpina3-in-human-prion-diseases
#17
S Vanni, F Moda, M Zattoni, E Bistaffa, E De Cecco, M Rossi, G Giaccone, F Tagliavini, S Haïk, J P Deslys, G Zanusso, J W Ironside, I Ferrer, G G Kovacs, G Legname
Prion diseases are fatal neurodegenerative disorders with sporadic, genetic or acquired etiologies. The molecular alterations leading to the onset and the spreading of these diseases are still unknown. In a previous work we identified a five-gene signature able to distinguish intracranially BSE-infected macaques from healthy ones, with SERPINA3 showing the most prominent dysregulation. We analyzed 128 suitable frontal cortex samples, from prion-affected patients (variant Creutzfeldt-Jakob disease (vCJD) n = 20, iatrogenic CJD (iCJD) n = 11, sporadic CJD (sCJD) n = 23, familial CJD (gCJD) n = 17, fatal familial insomnia (FFI) n = 9, Gerstmann-Sträussler-Scheinker syndrome (GSS)) n = 4), patients with Alzheimer disease (AD, n = 14) and age-matched controls (n = 30)...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28975068/longitudinal-white-matter-change-in-frontotemporal-dementia-subtypes-and-sporadic-late-onset-alzheimer-s-disease
#18
Fanny M Elahi, Gabe Marx, Yann Cobigo, Adam M Staffaroni, John Kornak, Duygu Tosun, Adam L Boxer, Joel H Kramer, Bruce L Miller, Howard J Rosen
BACKGROUND: Degradation of white matter microstructure has been demonstrated in frontotemporal lobar degeneration (FTLD) and Alzheimer's disease (AD). In preparation for clinical trials, ongoing studies are investigating the utility of longitudinal brain imaging for quantification of disease progression. To date only one study has examined sample size calculations based on longitudinal changes in white matter integrity in FTLD. OBJECTIVE: To quantify longitudinal changes in white matter microstructural integrity in the three canonical subtypes of frontotemporal dementia (FTD) and AD using diffusion tensor imaging (DTI)...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28968233/peripheral-versus-central-index-of-metabolic-dysfunction-and-associations-with-clinical-and-pathological-outcomes-in-alzheimer-s-disease
#19
Kelsey E McLimans, Joseph L Webb, Vellareddy Anantharam, Anumantha Kanthasamy, Auriel A Willette
BACKGROUND/OBJECTIVE: Insulin-like growth factor binding protein 2 (IGFBP-2) regulates blood glucose levels, facilitates hippocampal synaptic plasticity and may have a predictive value for Alzheimer's disease (AD) diagnosis. METHODS: IGFBP-2 levels were studied in plasma in 566 subjects and in cerebrospinal fluid (CSF) in 245 subjects across the AD spectrum from the Alzheimer's Disease Neuroimaging Initiative (ADNI). Variants in the IGFBP-2 gene were examined. Linear mixed modeling in SPSS tested main effects of IGFBP-2 and interactions with APOE4 on neurocognitive indices and biomarkers...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28813486/differentiating-between-subtypes-of-primary-progressive-aphasia-and-mild-cognitive-impairment-on-a-modified-version-of-the-frontal-behavioral-inventory
#20
Donna C Tippett, Carol B Thompson, Cornelia Demsky, Rajani Sebastian, Amy Wright, Argye E Hillis
Behavioral assessment has been investigated in frontotemporal lobar degeneration and Alzheimer's disease, but has not been explored extensively in subtypes of primary progressive aphasia (PPA). We explored the ability of a modified version of the Frontal Behavioral Inventory (FBI-mod) to discriminate between patients with distinct subtypes of PPA and patients with mild cognitive impairment (MCI). We hypothesized that individuals with nonfluent agrammatic PPA (nfaPPA) would have higher negative behavior scores than other groups and that individuals with semantic variant PPA (svPPA) would have higher disinhibition scores than other groups...
2017: PloS One
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