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frontotemporal lobar dementia

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https://www.readbyqxmd.com/read/29859866/latent-classes-of-mild-cognitive-impairment-are-associated-with-clinical-outcomes-and-neuropathology-analysis-of-data-from-the-national-alzheimer-s-coordinating-center
#1
John J Hanfelt, Limin Peng, Felicia C Goldstein, James J Lah
Given the importance of identifying prodromes of dementia with specific etiologies, we assessed whether seven latent classes of mild cognitive impairment (MCI), defined empirically based on cognitive, functional, and neuropsychiatric information at initial visit, are associated with distinct clinical outcomes and neuropathological features. We separated 6034 participants with a baseline diagnosis of MCI into seven latent classes using previously defined criteria. We found that these latent classes of MCI differed significantly in their clinical outcomes, survival time, and neuropathology...
May 31, 2018: Neurobiology of Disease
https://www.readbyqxmd.com/read/29845112/-anxa11-mutations-prevail-in-chinese-als-patients-with-and-without-cognitive-dementia
#2
Kang Zhang, Qing Liu, Keqiang Liu, Dongchao Shen, Hongfei Tai, Shi Shu, Qingyun Ding, Hanhui Fu, Shuangwu Liu, Zhili Wang, Xiaoguang Li, Mingsheng Liu, Xue Zhang, Liying Cui
Objective: To investigate the genetic contribution of ANXA11 , a gene associated with amyotrophic lateral sclerosis (ALS), in Chinese ALS patients with and without cognitive dementia. Methods: Sequencing all the coding exons of ANXA11 and intron-exon boundaries in 18 familial amyotrophic lateral sclerosis (FALS), 353 unrelated sporadic amyotrophic lateral sclerosis (SALS), and 12 Chinese patients with ALS-frontotemporal lobar dementia (ALS-FTD). The transcripts in peripheral blood generated from a splicing mutation were examined by reverse transcriptase PCR...
June 2018: Neurology. Genetics
https://www.readbyqxmd.com/read/29843237/serum-copper-is-not-altered-in-frontotemporal-lobar-degeneration
#3
Rosanna Squitti, Silvia Fostinelli, Mariacristina Siotto, Clarissa Ferrari, Giuliano Binetti, Luisa Benussi, Mauro Rongioletti, Roberta Ghidoni
Meta-analyses show copper dyshomeostasis in Alzheimer's disease. However, a study evaluating copper changes in other neurodegenerative forms of dementia has not yet been performed. In this study, we assessed copper, ceruloplasmin, copper not bound to ceruloplasmin, and copper to ceruloplasmin ratio in 85 patients affected by frontotemporal lobar degeneration (FTLD) and 55 healthy controls. Data were analyzed through multivariate ANOVA models taking into account age and sex as covariates and the stratification for FTLD variants, after calculating power analysis to ensure the reliability of the conclusions drawn...
May 18, 2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29807909/differential-neurotoxicity-related-to-tetracycline-transactivator-and-tdp-43-expression-in-conditional-tdp-43-mouse-model-of-frontotemporal-lobar-degeneration
#4
L Kukreja, R Shahidehpour, G Kim, J Keegan, K R Sadleir, T Russell, J Csernansky, M Mesulam, R J Vassar, L Wang, H Dong, C Geula
Frontotemporal lobar degeneration (FTLD) is among the most prevalent dementias of early-onset. Pathologically, FTLD presents with tauopathy or TAR DNA-binding protein 43 (TDP-43) proteinopathy. A biallelic mouse model of FTLD was produced on a mix FVB/129SVE background overexpressing wild-type human TDP-43 (hTDP-43) employing tetracycline transactivator (tTA), a system widely used in mouse models of neurological disorders. tTA activates hTDP-43 which is placed downstream of the tetracycline response element (TRE)...
May 28, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29760288/-neuropathologic-subtypes-of-frontotemporal-lobar-degeneration
#5
Mari Tada, Akiyoshi Kakita
Frontotemporal lobar degeneration (FTLD) is a heterogeneous disease entity encompassing a wide variety of histopathological features and genetic backgrounds. The last two decades have seen the discovery of causative genes and the identification of relevant proteins. The current histopathological classification is based on the major types of protein deposition in the brain, and most FTLD cases can be placed into one of three pathological subgroups: FTLD-tau, FTLD-TDP, and FTLD-FUS. Further sub-classification within each subgroup is based on the morphology of neuronal and glial inclusions and lesion distribution...
May 2018: Brain and Nerve, Shinkei Kenkyƫ No Shinpo
https://www.readbyqxmd.com/read/29746584/specific-serum-and-csf-microrna-profiles-distinguish-sporadic-behavioural-variant-of-frontotemporal-dementia-compared-with-alzheimer-patients-and-cognitively-healthy-controls
#6
Johannes Denk, Felix Oberhauser, Johannes Kornhuber, Jens Wiltfang, Klaus Fassbender, Matthias L Schroeter, Alexander E Volk, Janine Diehl-Schmid, Johannes Prudlo, Adrian Danek, Bernhard Landwehrmeyer, Martin Lauer, Markus Otto, Holger Jahn
Information on circulating miRNAs in frontotemporal lobar degeneration is very limited and conflicting results have complicated an interpretation in Alzheimer's disease thus far. In the present study we I) collected samples from multiple clinical centers across Germany, II) defined 3 homogenous patient groups with high sample sizes (bvFTD n = 48, AD n = 48 and cognitively healthy controls n = 44), III) compared expression levels in both CSF and serum samples and IV) detected a limited set of miRNAs by using a MIQE compliant protocol based on SYBR-green miRCURY assays that have proven reliable to generate reproducible results...
2018: PloS One
https://www.readbyqxmd.com/read/29742909/olfactory-testing-in-frontotemporal-dementia-a-literature-review
#7
Alessandro Tonacci, Lucia Billeci
Frontotemporal dementia (FTD) is a heterogeneous disorder featuring language impairment, personality changes, and executive defects, often due to the frontotemporal lobar degeneration (FTLD). Both FTD and FTLD are often associated with olfactory impairment, early biomarker for neurodegeneration, which can be evaluated with different techniques, among which low-cost olfactory tests are widely used. Therefore, we conducted a review of the literature focusing on papers published between January 1, 2007, and June 12, 2017, investigating the usefulness of olfactory testing in FTD/FTLD...
January 1, 2018: American Journal of Alzheimer's Disease and Other Dementias
https://www.readbyqxmd.com/read/29736698/clinical-utility-of-fdg-pet-for-the-differential-diagnosis-among-the-main-forms-of-dementia
#8
Peter J Nestor, Daniele Altomare, Cristina Festari, Alexander Drzezga, Jasmine Rivolta, Zuzana Walker, Femke Bouwman, Stefania Orini, Ian Law, Federica Agosta, Javier Arbizu, Marina Boccardi, Flavio Nobili, Giovanni Battista Frisoni
AIM: To assess the clinical utility of FDG-PET as a diagnostic aid for differentiating Alzheimer's disease (AD; both typical and atypical forms), dementia with Lewy bodies (DLB), frontotemporal lobar degeneration (FTLD), vascular dementia (VaD) and non-degenerative pseudodementia. METHODS: A comprehensive literature search was conducted using the PICO model to extract evidence from relevant studies. An expert panel then voted on six different diagnostic scenarios using the Delphi method...
May 7, 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29730823/the-correlation-between-striatal-and-cortical-binding-ratio-of-11-c-pib-pet-in-amyloid-uptake-positive-patients
#9
Julia Sauerbeck, Kazunari Ishii, Chisa Hosokawa, Hayato Kaida, Franziska T Scheiwein, Kohei Hanaoka, Axel Rominger, Matthias Brendel, Peter Bartenstein, Takamichi Murakami
PURPOSE: In subjects with amyloid deposition, striatal accumulation of 11 C-Pittsburgh compound B (PiB) demonstrated by positron emission tomography (PET) is related to the stage of Alzheimer's disease (AD). In this study, we investigated the correlation between striatal and cortical non-displaceable binding potential (BPND ). METHODS: Seventy-three subjects who complained of cognitive disturbance underwent dynamic PiB-PET studies and showed positive PiB accumulation were retrospectively selected...
May 5, 2018: Annals of Nuclear Medicine
https://www.readbyqxmd.com/read/29729314/the-human-mapt-locus-generates-circular-rnas
#10
Justin R Welden, Jacob van Doorn, Peter T Nelson, Stefan Stamm
The microtubule-associated protein Tau, generated by the MAPT gene is involved in dozens of neurodegenerative conditions ("tauopathies"), including Alzheimer's disease (AD) and frontotemporal lobar degeneration/frontotemporal dementia (FTLD/FTD). The pre-mRNA of MAPT is well studied and its aberrant pre-mRNA splicing is associated with frontotemporal dementia. Using a PCR screen of RNA from human brain tissues, we found that the MAPT locus generates circular RNAs through a backsplicing mechanism from exon 12 to either exon 10 or 7...
May 2, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29716643/tdp-43-pathology-in-anterior-temporal-pole-cortex-in-aging-and-alzheimer-s-disease
#11
Sukriti Nag, Lei Yu, Patricia A Boyle, Sue E Leurgans, David A Bennett, Julie A Schneider
TDP-43 pathology was investigated in the anterior temporal pole cortex (ATPC) and orbital frontal cortex (OFC), regions often degenerated in frontotemporal lobar degenerations (FTLD), in aging and Alzheimer's disease (AD). Diagnosis of dementia in the 1160 autopsied participants from 3 studies of community-dwelling elders was based on clinical evaluation and cognitive performance tests which were used to create summary measures of the five cognitive domains. Neuronal and glial TDP-43 cytoplasmic inclusions were quantitated in 8 brain regions by immunohistochemistry, and used in ANOVA and regression analyses...
May 1, 2018: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29704037/clinical-utility-of-fdg-pet-for-the-clinical-diagnosis-in-mci
#12
REVIEW
Javier Arbizu, Cristina Festari, Daniele Altomare, Zuzana Walker, Femke Bouwman, Jasmine Rivolta, Stefania Orini, Henryk Barthel, Federica Agosta, Alexander Drzezga, Peter Nestor, Marina Boccardi, Giovanni Battista Frisoni, Flavio Nobili
PURPOSE: We aim to report the quality of accuracy studies investigating the utility of [18 F]fluorodeoxyglucose (FDG)-PET in supporting the diagnosis of prodromal Alzheimer's Disease (AD), frontotemporal lobar degeneration (FTLD) and prodromal dementia with Lewy bodies (DLB) in mild cognitive impairment (MCI) subjects, and the corresponding recommendations made by a panel of experts. METHODS: Seven panellist, four from the European Association of Nuclear Medicine, and three from the European Academy of Neurology, produced recommendations taking into consideration the incremental value of FDG-PET, as added on clinical-neuropsychological examination, to ascertain the aetiology of MCI (AD, FTLD or DLB)...
April 27, 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29614680/novel-grn-mutations-in-alzheimer-s-disease-and-frontotemporal-lobar-degeneration
#13
Irene Piaceri, Daniele Imperiale, Enrico Ghidoni, Cristiana Atzori, Silvia Bagnoli, Camilla Ferrari, Silvana Ungari, Luca Ambrogio, Sandro Sorbi, Benedetta Nacmias
BACKGROUND: During the twentieth century, frontotemporal dementia (FTD) was often misdiagnosed, confused with Alzheimer's disease or psychiatric disorders, jeopardizing care and research. OBJECTIVE: To analyze the FTD genes in the DNA samples of patients belonging to families clinically classified as probable Alzheimer's disease (FAD) in the early 1990s and not carrying mutation in the three main genes linked to FAD (Presenilin 1, Presenilin 2, and Amyloid precursor protein)...
2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29578490/frontotemporal-dementia-and-chorea-associated-with-a-compound-heterozygous-trem2-mutation
#14
Veronica Redaelli, Ettore Salsano, Lara Colleoni, Paola Corbetta, Giovanni Tringali, Angelo Del Sole, Giorgio Giaccone, Giacomina Rossi
Frontotemporal dementia (FTD) is clinically characterized by behavioral changes, language impairment, and executive dysfunction. FTD usually belongs to the frontotemporal lobar degeneration (FTLD) disease group, and its familial forms are dominantly inherited and linked to a group of genes relevant to frontal and temporal brain pathology, such as MAPT, GRN, C9ORF72, TARDBP, CHMP2B, VCP, and FUS. However, FTD can also be associated with different clinical or pathological phenotypes caused by mutations in other genes, whose heredity can be dominant or recessive...
2018: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/29514947/clinical-value-of-neurofilament-and-phospho-tau-tau-ratio-in-the-frontotemporal-dementia-spectrum
#15
Lieke H H Meeter, Everard G Vijverberg, Marta Del Campo, Annemieke J M Rozemuller, Laura Donker Kaat, Frank Jan de Jong, Wiesje M van der Flier, Charlotte E Teunissen, John C van Swieten, Yolande A L Pijnenburg
OBJECTIVE: To examine the clinical value of neurofilament light chain (NfL) and the phospho-tau/total tau ratio (p/t-tau) across the entire frontotemporal dementia (FTD) spectrum in a large, well-defined cohort. METHODS: CSF NfL and p/t-tau levels were studied in 361 patients with FTD: 179 behavioral variant FTD, 17 FTD with motor neuron disease (FTD-MND), 36 semantic variant primary progressive aphasia (PPA), 19 nonfluent variant PPA, 4 logopenic variant PPA (lvPPA), 42 corticobasal syndrome, and 64 progressive supranuclear palsy...
April 3, 2018: Neurology
https://www.readbyqxmd.com/read/29512075/the-use-of-18-f-fdg-pet-in-the-diagnostic-workup-of-alzheimer-s-dementia
#16
Marion M Ortner
The diagnosis of dementia probably due to Alzheimer's disease is still primarily a clinical one. In cases that remain clinically unclear, however, biomarkers for amyloid deposition and neuronal injury can help to identify the underlying cause. One biomarker even for early neuronal injury in the stage of mild cognitive impairment is cerebral glucose hypometabolism measured by 18 F-FDG PET. Distinct patterns of hypometabolism can be seen, for example, in dementia due to Alzheimer's disease, frontotemporal lobar degeneration, and dementia with Lewy bodies...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29511772/-neurodegenerative-diseases
#17
Wolfgang Reith
Neurodegenerative diseases are sporadic and rare hereditary disorders of the central nervous system, which cause a slowly progressive loss of function of specific neuron populations and their connections. Severe impairments and care dependency can be the sequelae. Neurodegenerative disorders are diseases of older people; therefore, the demographic shift leads to an increase in the number of affected patients. Radiologists will also become more involved. For this reason important neurodegenerative diseases are presented in this article...
March 2018: Der Radiologe
https://www.readbyqxmd.com/read/29496136/minimal-neuropathologic-diagnosis-for-brain-banking-in-the-normal-middle-aged-and-aged-brain-and-in-neurodegenerative-disorders
#18
Irina Alafuzoff
Research on human brain diseases is currently often conducted on cell cultures and animals. Several questions however can only be addressed by studying human postmortem brain tissue. However, brain tissue obtained postmortem almost always displays pathology that is often related to the aging phenomenon. Thus, in order to be certain that the answers obtained are reliable, a systematic and thorough assessment of the brain tissue to be studied should be carried out. We are currently aware of several protein alterations that are found in middle-aged and aged brains that are obtained from neurologically unimpaired subjects...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29459769/exploring-the-genetics-and-non-cell-autonomous-mechanisms-underlying-als-ftld
#19
REVIEW
Hongbo Chen, Mark W Kankel, Susan C Su, Steve W S Han, Dimitry Ofengeim
Although amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, was first described in 1874, a flurry of genetic discoveries in the last 10 years has markedly increased our understanding of this disease. These findings have not only enhanced our knowledge of mechanisms leading to ALS, but also have revealed that ALS shares many genetic causes with another neurodegenerative disease, frontotemporal lobar dementia (FTLD). In this review, we survey how recent genetic studies have bridged our mechanistic understanding of these two related diseases and how the genetics behind ALS and FTLD point to complex disorders, implicating non-neuronal cell types in disease pathophysiology...
March 2018: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29453245/early-vs-late-age-at-onset-frontotemporal-dementia-and-frontotemporal-lobar-degeneration
#20
Sang Won Seo, Marie-Pierre Thibodeau, David C Perry, Alice Hua, Manu Sidhu, Isabel Sible, Jose Norberto S Vargas, Stephanie E Gaus, Gil D Rabinovici, Katherine D Rankin, Adam L Boxer, Joel H Kramer, Howard J Rosen, Maria Luisa Gorno-Tempini, Lea T Grinberg, Eric J Huang, Stephen J DeArmond, John Q Trojanowski, Bruce L Miller, William W Seeley
OBJECTIVE: To examine clinicopathologic correlations in early vs late age at onset frontotemporal dementia (FTD) and frontotemporal lobar degeneration (FTLD). METHODS: All patients were clinically evaluated and prospectively diagnosed at the UCSF Memory and Aging Center. Two consecutive series were included: (1) patients with a clinically diagnosed FTD syndrome who underwent autopsy (cohort 1) and (2) patients with a primary pathologic diagnosis of FTLD, regardless of the clinical syndrome (cohort 2)...
March 20, 2018: Neurology
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