... 11 5 2021 Magnetic resonance imaging Diffusion tensor imaging Tau Frontotemporal dementia Animal models Alzheimer’s disease (AD), progressive supranuclear palsy, and corticobasal degeneration and frontotemporal dementia including frontotemporal dementia with Parkinsonism linked...

...(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements. Amyotrophic lateral sclerosis C9orf72 repeat expansion Frontotemporal dementia Huntington disease genetic testing...

...Carlo Wilke; Frank Gillardon; Christian Deuschle; Markus A. Hobert; Iris E. Jansen; Florian G. Metzger; Peter Heutink; Thomas Gasser; Walter Maetzler; Cornelis Blauwendraat; Matthis Synofzik Background and Objective: Reduced progranulin levels are a hallmark of frontotemporal dementia (FTD) caused...

...Andrea Pilotto; Federica Rossi; Fabrizio Rinaldi; Silvia Compostella; Maura Cosseddu; Barbara Borroni; Massimiliano Filosto; Alessandro Padovani Background: Behavioral and cognitive impairment are common in amyotrophic lateral sclerosis (ALS) and represent a continuum with frontotemporal dementia...

... the elderly. The GGGGCC hexanucleotide expansion mutation at the C9orf72 locus has been identified as a major cause of amyotrophic lateral sclerosis and frontotemporal dementia, raising the question of whether this mutation is a factor in DLB. Furthermore, a small number of clinically diagnosed DLB patients...

... symptom of frontotemporal dementia (FTD) and has been linked to frontal-subcortical dopaminergic system dysfunction. No pharmacological therapy has been approved for the treatment of apathy, but, on the basis of its physiopathological mechanism, we suspected that increasing prefrontal dopaminergic...

...Aurélia Vernay; François Sellal; Frédérique René The behavioral variant of frontotemporal dementia (bvFTD) is a neurodegenerative disease affecting people in their early sixties, characterized by dramatic changes in individual and social behavior. Despite the heterogeneity in the presentation...

... to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements. Amyotrophic lateral sclerosis Amyloid β protein Atrophy Frontotemporal dementia Frontotemporal lobar degeneration Motor neuron diseases Neurofibrillary tangles Tau...

... for Neurodegenerative Disorders, University of Milan, Milan, Italy Key Words Frontotemporal lobar degeneration Frontotemporal dementia Progranulin MAPT haplotype VEGF polymorphisms APOE Abstract Background: Ruling out predictors of survival in frontotemporal lobar degeneration (FTLD) is a clinical challenge...

...Jonathan D. Rohrer; Dominic Paviour; Jana Vandrovcova; John Hodges; Rohan de Silva; Martin N. Rossor Background: MAPT mutations are associated with disorders within the frontotemporal lobar degeneration spectrum. The usual presenting syndrome is behavioural variant frontotemporal dementia, although...

...Dominik Paquet; Bettina Schmid; Christian Haass Our ageing society is confronted with a dramatic increase in patients suffering from tauopathies such as Alzheimer’s disease, frontotemporal dementia and others. Typical neuropathological lesions including tangles composed of hyperphosphorylated tau...

...-term cultures. To determine tau toxicity, K369I mutant tau mice were established as a model of frontotemporal dementia, analyzed biochemically and compared with human diseased brain. Results: Oligomeric and fibrillar Aβ42 were both toxic, although to different degrees. Human amylin shared toxicity...

...Ali Jawaid; Rosa Rademakers; Joseph S. Kass; Yogeshwar Kalkonde; Paul E. Schulz Frontotemporal lobar degeneration with TAR-DNA-binding protein inclusions (FTLD-TDP) is the most common pathological subtype of frontotemporal dementia (FTD). Mutations leading to a loss of function in the progranulin...

...) deposits. Lewy body diseases (LBD), such as sporadic Parkinson disease (PD) and dementia with Lewy bodies (DLB), show aSyn-positive deposits in neurons, neurites, glia, and presynaptic terminals, while frontotemporal dementias present tau-positive and tau-negative, ubiquitin- and TDP-43-positive neuronal...

... categories, i.e. stress response and metabolism. Among the proteins identified was valosin-containing protein (VCP), a component of the quality control system during endoplasmic reticulum stress. Mutations in VCP have recently been linked to frontotemporal dementia. Conclusion: Our data support the mitosis...

...Christian Wider; Zbigniew K. Wszolek Genetic studies have led to major discoveries in the pathogenesis of various neurodegenerative diseases. Ubiquitin-positive familial frontotemporal dementia was recently found to be caused by mutations in the progranulin gene (PGRN), and the major constituent...

...Fereshteh Sedaghat; Anna Gotzamani-Psarrakou; Eleni Dedousi; Marianthi Arnaoutoglou; Kyriakos Psarrakos; Ioannis Baloyannis; Athanasios S. Dimitriadis; Stavros J. Baloyannis Extrapyramidal symptoms are observed in frontotemporal dementia (FTD). 123 I-FP-CIT (DaT scan) single photon emission...

... Alzheimer’s disease. The involvement of tau in neurodegeneration has been clarified by the identification of genetic mutations in the tau gene in cases with familial frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). Although the mechanism through which tau mutations lead to neuronal...

...; John Hardy A nonsense/protein chain-terminating mutation in the CHMP2B gene has recently been reported as a cause of frontotemporal dementia (FTD) in the single large family known to show linkage to chromosome 3. Screening for mutations in this gene in a large series of FTD families and individual...

... including Alzheimer’s disease (AD), frontotemporal dementia (FTD) and Parkinson’s disease (PD). A single nucleotide polymorphism in the STH gene has been suggested to be involved in sporadic AD and is in complete linkage disequilibrium with the TAU haplotype H1. Objective: A case-control study was performed...