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https://www.readbyqxmd.com/read/28805003/protein-astrogliopathies-in-human-neurodegenerative-diseases-and-aging
#1
Gabor G Kovacs, Virginia M Lee, John Q Trojanowski
Neurodegenerative diseases are characterized by progressive dysfunction and loss of neurons associated with depositions of pathologically altered proteins showing hierarchical involvement of brain regions. The role of astrocytes in the pathogenesis of neurodegenerative diseases is explored as contributors to neuronal degeneration or neuroprotection pathways, and also as potential mediators of the transcellular spreading of disease-associated proteins. Protein astrogliopathy (PAG), including deposition of amyloid-β, prion protein, tau, α-synuclein, and very rarely transactive response DNA-binding protein 43 (TDP-43) is not unprecedented or unusual in neurodegenerative diseases...
September 2017: Brain Pathology
https://www.readbyqxmd.com/read/28803444/atypical-parkinsonian-syndromes-a-general-neurologist-s-perspective
#2
REVIEW
Angela B Deutschländer, Owen A Ross, Dennis W Dickson, Zbigniew K Wszolek
The differential diagnosis of atypical parkinsonian syndromes is challenging. These severe and often rapidly progressive neurodegenerative disorders are clinically heterogeneous and show significant phenotypic overlap. Here we review clinical, imaging, neuropathologic and genetic features of multiple system atrophy (MSA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and frontotemporal lobar degeneration (FTLD). The terms CBD and FTLD refer to pathologically confirmed cases of corticobasal syndrome (CBS) and frontotemporal dementia (FTD)...
August 12, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28801064/can-different-mechanisms-underpin-the-geschwind-syndrome-in-temporal-lobe-epilepsy-and-in-temporal-lobe-variant-of-frontotemporal-degeneration
#3
Guido Gainotti
No abstract text is available yet for this article.
July 14, 2017: Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
https://www.readbyqxmd.com/read/28800332/cerebral-oxidative-stress-and-microvasculature-defects-in-tnf-%C3%AE-expressing-transgenic-and-porphyromonas-gingivalis-infected-apoe-mice
#4
Farheen Rokad, Ryan Moseley, Rowan S Hardy, Sasanka Chukkapalli, StJohn Crean, Lakshmyya Kesavalu, Sim K Singhrao
The polymicrobial dysbiotic subgingival biofilm microbes associated with periodontal disease appear to contribute to developing pathologies in distal body sites, including the brain. This study examined oxidative stress, in the form of increased protein carbonylation and oxidative protein damage, in the tumor necrosis factor-α (TNF-α) transgenic mouse that models inflammatory TNF-α excess during bacterial infection; and in the apolipoprotein knockout (ApoE-/-) mouse brains, following Porphyromonas gingivalis gingival monoinfection...
August 8, 2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28795386/dementia-caregiver-burden-a-research-update-and-critical-analysis
#5
REVIEW
Sheung-Tak Cheng
PURPOSE OF REVIEW: This article provides an updated review of the determinants of caregiver burden and depression, with a focus on care demands and especially the differential effects of various neuropsychiatric symptoms or symptom clusters. Moreover, studies on caregivers for frontotemporal and Lewy body dementias were referred to in order to identify differences and similarities with the mainstream literature based largely on Alzheimer caregivers. RECENT FINDINGS: As a group, neuropsychiatric symptoms are most predictive of caregiver burden and depression regardless of dementia diagnosis, but the effects appear to be driven primarily by disruptive behaviors (e...
August 10, 2017: Current Psychiatry Reports
https://www.readbyqxmd.com/read/28793933/can-cognitive-assessment-really-discriminate-early-stages-of-alzheimer-s-and-behavioural-variant-frontotemporal-dementia-at-initial-clinical-presentation
#6
Sophia Reul, Hubertus Lohmann, Heinz Wiendl, Thomas Duning, Andreas Johnen
BACKGROUND: Neuropsychological testing is considered crucial for differential diagnosis of Alzheimer's disease (AD) and behavioural variant frontotemporal dementia (bvFTD). In-depth neuropsychological assessment revealed specific dysfunctions in the two dementia syndromes. However, a significant overlap of cognitive impairments exists in early disease stages. We questioned whether a standard neuropsychological assessment at initial clinical presentation can delineate patients with AD versus bvFTD...
August 9, 2017: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/28790215/-a-case-of-ruptured-distal-anterior-choroidal-artery-aneurysm-associated-with-a-twig-like-middle-cerebral-artery-treated-with-single-stage-aneurysm-clipping-and-sta-mca-double-anastomoses-in-the-acute-phase
#7
Toshimoto Seno, Kanehisa Kohno, Hideo Tanaka, Shinji Iwata, Shinya Fukumoto, Haruhisa Ichikawa, Noriyuki Fumoto, Akihiro Inoue, Saya Ozaki, Yawara Nakamura, Shohei Kohno, Shiro Ohue
We report the case of a patient who has progressed well over 5 years following single-stage aneurysm clipping and superficial temporal artery-middle cerebral artery(STA-MCA)double anastomoses in the acute phase, for a ruptured distal anterior choroidal artery(AChA)aneurysm accompanied by a twig-like MCA. The patient was a 49-year-old female who developed a sudden severe headache and disturbance of consciousness due to subarachnoid hemorrhage and intraventricular hemorrhage(IVH). Cerebral angiography showed a right twig-like MCA associated with an abnormal vascular network and a ruptured aneurysm in the distal AChA...
August 2017: No Shinkei Geka. Neurological Surgery
https://www.readbyqxmd.com/read/28790177/phosphorylation-of-the-fus-low-complexity-domain-disrupts-phase-separation-aggregation-and-toxicity
#8
Zachary Monahan, Veronica H Ryan, Abigail M Janke, Kathleen A Burke, Shannon N Rhoads, Gül H Zerze, Robert O'Meally, Gregory L Dignon, Alexander E Conicella, Wenwei Zheng, Robert B Best, Robert N Cole, Jeetain Mittal, Frank Shewmaker, Nicolas L Fawzi
Neuronal inclusions of aggregated RNA-binding protein fused in sarcoma (FUS) are hallmarks of ALS and frontotemporal dementia subtypes. Intriguingly, FUS's nearly uncharged, aggregation-prone, yeast prion-like, low sequence-complexity domain (LC) is known to be targeted for phosphorylation. Here we map in vitro and in-cell phosphorylation sites across FUS LC We show that both phosphorylation and phosphomimetic variants reduce its aggregation-prone/prion-like character, disrupting FUS phase separation in the presence of RNA or salt and reducing FUS propensity to aggregate...
August 8, 2017: EMBO Journal
https://www.readbyqxmd.com/read/28776681/another-piece-in-the-progranulin-puzzle-special-binding-between-progranulin-and-prosaposin-creates-additional-lysosomal-access-an-editorial-comment-for-the-interaction-between-progranulin-and-prosaposin-is-mediated-by-granulins-and-the-linker-region-between
#9
EDITORIAL
Philip Van Damme
Loss-of-function mutations in the gene encoding the growth factor progranulin cause degeneration of the ageing brain in a dose-dependent manner. While heterozygous mutations result in adult onset frontotemporal dementia, the much rarer homozygous null mutations cause an early onset lysosomal storage disorder. A better understanding of the biology of progranulin in the central nervous system is needed to find solutions for these incurable diseases. This Editorial highlights a study by Zhou et al. in the current issue of the Journal of Neurochemistry, in which the authors provide data that are a step towards this goal...
August 4, 2017: Journal of Neurochemistry
https://www.readbyqxmd.com/read/28766957/immunohistochemical-detection-of-c9orf72-protein-in-frontotemporal-lobar-degeneration-and-motor-neurone-disease-patterns-of-immunostaining-and-an-evaluation-of-commercial-antibodies
#10
Yvonne S Davidson, Andrew C Robinson, Sara Rollinson, Stuart Pickering-Brown, Shangxi Xiao, Janice Robertson, David M A Mann
We have employed as 'gold standards' two in-house, well-characterised and validated polyclonal antibodies, C9-L and C9-S, which detect the longer and shorter forms of C9orf72, and have compared seven other commercially available antibodies with these in order to evaluate the utility of the latter as credible tools for the demonstration of C9orf72. C9-L and C9-S antibodies immunostained cytoplasmic 'speckles', and the nuclear membrane, respectively, in cerebellar Purkinje cells of the cerebellum in patients with behavioural variant frontotemporal dementia (bvFTD) with amyotrophic lateral sclerosis (ALS), and in patients with ALS alone...
August 2, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28756984/a-55-year-old-female-with-leukoencephalopathy-with-cerebral-calcifications-and-cysts-case-report-and-radiopathologic-description
#11
Jorge Novo, Diana Lin, Megan Shanks, Mehmet Kocak, Leonidas Arvanitis
Adult-onset leukoencephalopathies with increased cerebral volume can present a potentially challenging diagnosis for the pathologist. We present the case of a patient with a rare adult-onset disease called Leukoencephalopathy with cerebral Calcifications and Cysts (LCC). A 55-year-old woman with a history of morning headaches, mild memory loss, diabetes, and hypertension presented to the emergency department with acute onset altered mental status. CT scan revealed multiple small hypodense lesions in the white matter with calcifications in the bilateral cerebral hemispheres, basal ganglia, pons, and cerebellar hemispheres...
July 6, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28754988/zinc-binding-to-rna-recognition-motif-of-tdp-43-induces-the-formation-of-amyloid-like-aggregates
#12
Cyrille Garnier, François Devred, Deborah Byrne, Rémy Puppo, Andrei Yu Roman, Soazig Malesinski, Andrey V Golovin, Régine Lebrun, Natalia N Ninkina, Philipp O Tsvetkov
Aggregation of TDP-43 (transactive response DNA binding protein 43 kDa) is a hallmark of certain forms of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Moreover, intracellular TDP-43-positive inclusions are often found in other neurodegenerative diseases. Recently it was shown that zinc ions can provoke the aggregation of endogenous TDP-43 in cells, allowing to assume a direct interaction of TDP-43 with zinc ions. In this work, we investigated zinc binding to the 102-269 TDP-43 fragment, which comprise the two RNA recognition motifs...
July 28, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28751743/anti-ampa-glua3-antibodies-in-frontotemporal-dementia-a-new-molecular-target
#13
B Borroni, J Stanic, C Verpelli, M Mellone, E Bonomi, A Alberici, P Bernasconi, L Culotta, E Zianni, S Archetti, M Manes, S Gazzina, R Ghidoni, L Benussi, C Stuani, M Di Luca, C Sala, E Buratti, A Padovani, F Gardoni
Frontotemporal Dementia (FTD) is a neurodegenerative disorder mainly characterised by Tau or TDP43 inclusions. A co-autoimmune aetiology has been hypothesised. In this study, we aimed at defining the pathogenetic role of anti-AMPA GluA3 antibodies in FTD. Serum and cerebrospinal fluid (CSF) anti-GluA3 antibody dosage was carried out and the effect of CSF with and without anti-GluA3 antibodies was tested in rat hippocampal neuronal primary cultures and in differentiated neurons from human induced pluripotent stem cells (hiPSCs)...
July 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28750498/emerging-clinical-issues-and-multivariate-analyses-in-pet-investigations
#14
Javier Arbizu, Alessandro Giuliani, Jaime G Perez-Larraya, Mario Riverol, Cathrine Jonsson, Berta García-García, Maribel Morales, Laura Imaz, Marco Pagani
PET using 18F-2-fluoro-2-deoxy-D-glucose (FDG-PET) has been gradually introduced in the diagnostic clinical criteria of the most prevalent neurodegenerative diseases. Moreover, an increasing amount of literature have shown that the information provided by FDG-PET enhances the sensitivity of standard imaging biomarkers in less frequent disorders in which an early differential diagnosis can be of paramount relevance for patient management and outcome. Therefore emerging uses of FDG-PET may be important in prion diseases, autoimmune encephalitis and amyotrophic lateral sclerosis...
July 27, 2017: Quarterly Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28749476/the-wide-genetic-landscape-of-clinical-frontotemporal-dementia-systematic-combined-sequencing-of-121-consecutive-subjects
#15
Cornelis Blauwendraat, Carlo Wilke, Javier Simón-Sánchez, Iris E Jansen, Anika Reifschneider, Anja Capell, Christian Haass, Melissa Castillo-Lizardo, Saskia Biskup, Walter Maetzler, Patrizia Rizzu, Peter Heutink, Matthis Synofzik
PurposeTo define the genetic spectrum and relative gene frequencies underlying clinical frontotemporal dementia (FTD).MethodsWe investigated the frequencies and mutations in neurodegenerative disease genes in 121 consecutive FTD subjects using an unbiased, combined sequencing approach, complemented by cerebrospinal fluid Aβ1-42 and serum progranulin measurements. Subjects were screened for C9orf72 repeat expansions, GRN and MAPT mutations, and, if negative, mutations in other neurodegenerative disease genes, by whole-exome sequencing (WES) (n = 108), including WES-based copy-number variant (CNV) analysis...
July 27, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28749089/cognitive-deficits-in-patients-with-neuropsychiatric-symptoms-a-comparative-study-between-behavioral-variant-frontotemporal-dementia-and-primary-psychiatric-disorders
#16
Everard G B Vijverberg, Sigfried Schouws, Paul David Meesters, Esmée Verwijk, Hannie Comijs, Ted Koene, Charlotte Schreuder, Aartjan Beekman, Philip Scheltens, Max Stek, Yolande Pijnenburg, Annemieke Dols
OBJECTIVE: To compare neuropsychological profiles in behavioral variant frontotemporal dementia (bvFTD) with its most common primary psychiatric differential diagnoses, major depressive disorder (MDD), bipolar disorder (BD), and schizophrenia, in older patients with active symptoms. METHODS: We included patients from different cohorts with MDD (DSM-IV-TR: 296.20-296.23, 296.30-296.33; n = 42; mean ± SD age, 72.0 ± 8.0 years; female = 57.1%) included from 2002 to 2007, noneuthymic BD (DSM-IV-TR: 296...
July 25, 2017: Journal of Clinical Psychiatry
https://www.readbyqxmd.com/read/28748674/prognosis-of-patients-with-behavioral-variant-frontotemporal-dementia-who-have-focal-versus-diffuse-frontal-atrophy
#17
Jin San Lee, Na Yeon Jung, Young Kyoung Jang, Hee Jin Kim, Sang Won Seo, Juyoun Lee, Yeo Jin Kim, Jae Hong Lee, Byeong C Kim, Kyung Won Park, Soo Jin Yoon, Jee H Jeong, Sang Yun Kim, Seung Hyun Kim, Eun Joo Kim, Key Chung Park, David S Knopman, Duk L Na
BACKGROUND AND PURPOSE: Only a few studies have investigated the relationship between different subtypes and disease progression or prognosis in patients with behavioral variant frontotemporal dementia (bvFTD). Since a localized injury often produces more focal signs than a diffuse injury, we hypothesized that the clinical characteristics differ between patients with bvFTD who show diffuse frontal lobe atrophy (D-type) on axial magnetic resonance imaging (MRI) scans versus those with focal or circumscribed frontal lobe atrophy (F-type)...
July 2017: Journal of Clinical Neurology
https://www.readbyqxmd.com/read/28747446/transcranial-magnetic-stimulation-distinguishes-alzheimer-disease-from-frontotemporal-dementia
#18
Alberto Benussi, Francesco Di Lorenzo, Valentina Dell'Era, Maura Cosseddu, Antonella Alberici, Salvatore Caratozzolo, Maria Sofia Cotelli, Anna Micheli, Luca Rozzini, Alessandro Depari, Alessandra Flammini, Viviana Ponzo, Alessandro Martorana, Carlo Caltagirone, Alessandro Padovani, Giacomo Koch, Barbara Borroni
OBJECTIVE: To determine whether a transcranial magnetic stimulation (TMS) multiparadigm approach can be used to distinguish Alzheimer disease (AD) from frontotemporal dementia (FTD). METHODS: Paired-pulse TMS was used to investigate short-interval intracortical inhibition (SICI) and facilitation (ICF), long-interval intracortical inhibition, and short-latency afferent inhibition (SAI) to measure the activity of different intracortical circuits in patients with AD, patients with FTD, and healthy controls (HC)...
July 26, 2017: Neurology
https://www.readbyqxmd.com/read/28745408/a-case-of-frontotemporal-dementia-with-sexual-disinhibition-controlled-by-aripiprazole
#19
Hiroshi Nomoto, Yoichiro Matsubara, Yosuke Ichimiya, Heii Arai
No abstract text is available yet for this article.
July 26, 2017: Psychogeriatrics: the Official Journal of the Japanese Psychogeriatric Society
https://www.readbyqxmd.com/read/28745227/frontotemporal-lobar-degeneration-review-and-update-for-clinical-neurologists
#20
Isabel Hernández, Maria-Victoria Fernández, Lluis Tàrraga, Mercè Boada, Agustín Ruiz
BACKGROUND: Frontotemporal Dementia (FTD) is a heterogeneous group of disorders and the second most frequent cause of early onset dementia making it the highest number of inherited cases. METHODS: FTL is characterized by considerable variability in clinical, genetic and histopathologic features. Patients may present symptoms ranging from behavioural disturbances to different language disorders, with or without motor neuron disorders or associated parkinsonism. Atrophy in frontal and temporal lobes is the most relevant radiological finding...
July 25, 2017: Current Alzheimer Research
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