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Frontotemporal Dementia and Frontotemporal Lobar Degeneration

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In vivo staging of frontotemporal lobar degeneration TDP-43 type C pathology.

Martina Bocchetta, Maria Del Mar Iglesias Espinosa, Tammaryn Lashley, Jason D Warren, Jonathan D Rohrer,

BACKGROUND:TDP-43 type C is one of the pathological forms of frontotemporal lobar degeneration (FTLD) and mainly associated clinically with the semantic variant of primary progressive aphasia (svPPA). We aimed to define in vivo the sequential pattern of neuroanatomical involvement in a cohort of patients with FTLD-TDP type C pathology. METHODS:We ... Read more >>

Alzheimers Res Ther (Alzheimer's research & therapy)
[2020, 12(1):34]

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Nasal vaccine delivery attenuates brain pathology and cognitive impairment in tauopathy model mice.

Hiroki Takeuchi, Keiko Imamura, Bin Ji, Kayoko Tsukita, Takako Enami, Keizo Takao, Tsuyoshi Miyakawa, Masato Hasegawa, Naruhiko Sahara, Nobuhisa Iwata, Makoto Inoue, Hideo Hara, Takeshi Tabira, Maiko Ono, John Q Trojanowski, Virginia M-Y Lee, Ryosuke Takahashi, Tetsuya Suhara, Makoto Higuchi, Haruhisa Inoue,

Pathological aggregates of tau proteins accumulate in the brains of neurodegenerative tauopathies including Alzheimer's disease and frontotemporal lobar degeneration (FTLD-tau). Although immunotherapies of these disorders against tau are emerging, it is unknown whether nasal delivery, which offers many benefits over traditional approaches to vaccine administration, is effective or not for ... Read more >>

NPJ Vaccines (NPJ Vaccines)
[2020, 5:28]

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Nasal vaccine delivery attenuates brain pathology and cognitive impairment in tauopathy model mice.

Hiroki Takeuchi, Keiko Imamura, Bin Ji, Kayoko Tsukita, Takako Enami, Keizo Takao, Tsuyoshi Miyakawa, Masato Hasegawa, Naruhiko Sahara, Nobuhisa Iwata, Makoto Inoue, Hideo Hara, Takeshi Tabira, Maiko Ono, John Q. Trojanowski, Virginia M.-Y. Lee, Ryosuke Takahashi, Tetsuya Suhara, Makoto Higuchi, Haruhisa Inoue,

Pathological aggregates of tau proteins accumulate in the brains of neurodegenerative tauopathies including Alzheimer’s disease and frontotemporal lobar degeneration (FTLD-tau). Although immunotherapies of these disorders against tau are emerging, it is unknown whether nasal delivery, which offers many benefits over traditional approaches to vaccine administration, is effective or not for ... Read more >>

NPJ Vaccines (NPJ Vaccines)
[2020, 5:]

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Reappraisal of the anatomical spreading and propagation hypothesis about TDP-43 aggregation in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.

Yuichi Riku,

Neuronal inclusion of transactivation response DNA-binding protein 43 kDa (TDP-43) is known to be a pathologic hallmark of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). TDP-43, which is physiologically a nuclear protein, is mislocalized from the nucleus and aggregated within the cytoplasm of affected neurons in ALS and FTLD ... Read more >>

Neuropathology (Neuropathology : official journal of the Japanese Society of Neuropathology)
[2020, :]

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Mixed neuropathology in frontotemporal lobar degeneration.

Catherine Pennington, Luca Marini, Elizabeth Coulthard, Seth Love,

Aim: Frontotemporal lobar degeneration (FTLD) is a significant cause of dementia in mid-life and older adults. The extent of interactions between FTLD and other neurodegenerative pathologies is unclear. We reviewed the occurrences of mixed pathology in cases of neuropathologically diagnosed FTLD from the UK Brain Bank Network. Materials and methods: ... Read more >>

Amyotroph Lateral Scler Frontotemporal Degener (Amyotrophic lateral sclerosis & frontotemporal degeneration)
[2020, :1-8]

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Anti-Cholinergic Derangement of Cortical Metabolism on 18F-FDG PET in a Patient with Frontotemporal Lobar Degeneration Dementia: A Case of the TREDEM Registry.

Maurizio Gallucci, Claudia Pallucca, Maria Elena Di Battista, Cristina Bergamelli, Vittorio Fiore, Franco Boccaletto, Michele Fiorini, Daniela Perra, Gianluigi Zanusso, Chiara Fenoglio, Maria Serpente, Daniela Galimberti, Laura Bonanni,

We present the case of a patient with an atypical course of frontotemporal lobar degeneration (FTLD) complicated by the use of an anticholinergic drug. A 70-year-old patient, followed by psychiatrists for depression and behavioral disorders, received a diagnosis of dementia with Lewy bodies (DLB) at another Center due to auditory ... Read more >>

J. Alzheimers Dis. (Journal of Alzheimer's disease : JAD)
[2020, :]

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Cerebrospinal fluid phospho-tau T217 outperforms T181 as a biomarker for the differential diagnosis of Alzheimer's disease and PET amyloid-positive patient identification.

Nicolas R Barthélemy, Randall J Bateman, Christophe Hirtz, Philippe Marin, François Becher, Chihiro Sato, Audrey Gabelle, Sylvain Lehmann,

BACKGROUND:Cerebrospinal fluid biomarker profiles characterized by decreased amyloid-beta peptide levels and increased total and phosphorylated tau levels at threonine 181 (pT181) are currently used to discriminate between Alzheimer's disease and other neurodegenerative diseases. However, these changes are not entirely specific to Alzheimer's disease, and it is noteworthy that other phosphorylated ... Read more >>

Alzheimers Res Ther (Alzheimer's research & therapy)
[2020, 12(1):26]

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Conditional depletion of Fus in oligodendrocytes leads to motor hyperactivity and increased myelin deposition associated with Akt and cholesterol activation.

Kelly M Guzman, Lauren E Brink, Guillermo Rodriguez-Bey, Richard J Bodnar, Lisha Kuang, Bin Xing, Mara Sullivan, Hyun J Park, Erik Koppes, Haining Zhu, Quasar Padiath, Franca Cambi,

Fused in sarcoma (FUS) is a predominantly nuclear multifunctional RNA/DNA-binding protein that regulates multiple aspects of gene expression. FUS mutations are associated with familial amyotrophic lateral sclerosis (fALS) and frontotemporal lobe degeneration (FTLD) in humans. At the molecular level, the mutated FUS protein is reduced in the nucleus but accumulates ... Read more >>

Glia (Glia)
[2020, :]

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Speech and Language Presentations of FTLD-TDP Type B Neuropathology.

Daniel J Lee, Eileen H Bigio, Emily J Rogalski, M-Marsel Mesulam,

Four right-handed patients who presented with an isolated impairment of speech or language had transactive response DNA-binding protein of 43 kDa (TDP-43) type B pathology. Comportment and pyramidal motor function were preserved at presentation. Three of the cases developed axial rigidity and oculomotor findings late in their course with no ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(3):277-283]

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Diverse, evolving conformer populations drive distinct phenotypes in frontotemporal lobar degeneration caused by the same MAPT-P301L mutation.

Nathalie Daude, Chae Kim, Sang-Gyun Kang, Ghazaleh Eskandari-Sedighi, Tracy Haldiman, Jing Yang, Shelaine C Fleck, Erik Gomez-Cardona, Zhuang Zhuang Han, Sergi Borrego-Ecija, Serene Wohlgemuth, Olivier Julien, Holger Wille, Laura Molina-Porcel, Ellen Gelpi, Jiri G Safar, David Westaway,

Tau protein accumulation is a common denominator of major dementias, but this process is inhomogeneous, even when triggered by the same germline mutation. We considered stochastic misfolding of human tau conformers followed by templated conversion of native monomers as an underlying mechanism and derived sensitive conformational assays to test this ... Read more >>

Acta Neuropathol. (Acta neuropathologica)
[2020, :]

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Neuroinflammation and protein aggregation co-localize across the frontotemporal dementia spectrum.

W Richard Bevan-Jones, Thomas E Cope, P Simon Jones, Sanne S Kaalund, Luca Passamonti, Kieren Allinson, Oliver Green, Young T Hong, Tim D Fryer, Robert Arnold, Jonathan P Coles, Franklin I Aigbirhio, Andrew J Larner, Karalyn Patterson, John T O'Brien, James B Rowe,

The clinical syndromes of frontotemporal dementia are clinically and neuropathologically heterogeneous, but processes such as neuroinflammation may be common across the disease spectrum. We investigated how neuroinflammation relates to the localization of tau and TDP-43 pathology, and to the heterogeneity of clinical disease. We used PET in vivo with (i) ... Read more >>

Brain (Brain : a journal of neurology)
[2020, 143(3):1010-1026]

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Imaging Biomarkers for Neurodegeneration in Presymptomatic Familial Frontotemporal Lobar Degeneration.

Qin Chen, Kejal Kantarci,

Frontotemporal lobar degeneration (FTLD) is a neurodegenerative disorder characterized by behavioral changes, language abnormality, as well as executive function deficits and motor impairment. In about 30-50% of FTLD patients, an autosomal dominant pattern of inheritance was found with major mutations in the MAPT, GRN, and the C9orf72 repeat expansion. These ... Read more >>

Front Neurol (Frontiers in Neurology)
[2020, 11:80]

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Biomolecular condensates in cell biology and virology: phase-separated membraneless organelles (MLOs).

Pravin B Sehgal, Jenna Westley, Kenneth M Lerea, Susan DiSenso-Browne, Joseph D Etlinger,

Membraneless organelles (MLOs) in the cytoplasm and nucleus in the form of 2D and 3D phase-separated biomolecular condensates are increasingly viewed as critical in regulating diverse cellular functions. These functions include cell signaling, immune synapse function, nuclear transcription, RNA splicing and processing, mRNA storage and translation, virus replication and maturation, ... Read more >>

Anal. Biochem. (Analytical biochemistry)
[2020, :113691]

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Role of CNC1 gene in TDP-43 aggregation-induced oxidative stress-mediated cell death in S. cerevisiae model of ALS

Vidhya Bharathi, Amandeep Girdhar, Basant Patel,

ABSTRACT TDP-43 is a multi-functional ribonucleoprotein that is also found deposited as hyper-phosphorylated and ubiquitinated TDP-43 inclusions in the brain and spinal cord of the patients of the motor neuron diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). Till date, how the cell death ensues is not fully ... Read more >>

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β-Arrestin2 oligomers impair the clearance of pathological tau and increase tau aggregates.

Jung-A A Woo, Tian Liu, Cenxiao C Fang, Maria A Castaño, Teresa Kee, Ksenia Yrigoin, Yan Yan, Sara Cazzaro, Jenet Matlack, Xinming Wang, Xingyu Zhao, David E Kang, Stephen B Liggett,

Multiple G protein-coupled receptors (GPCRs) are targets in the treatment of dementia, and the arrestins are common to their signaling. β-Arrestin2 was significantly increased in brains of patients with frontotemporal lobar degeneration (FTLD-tau), a disease second to Alzheimer's as a cause of dementia. Genetic loss and overexpression experiments using genetically ... Read more >>

Proc. Natl. Acad. Sci. U.S.A. (Proceedings of the National Academy of Sciences of the United States of America)
[2020, 117(9):5006-5015]

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Liquid-Liquid Phase Separation and Its Mechanistic Role in Pathological Protein Aggregation.

W Michael Babinchak, Witold K Surewicz,

Liquid-liquid phase separation (LLPS) of proteins underlies the formation of membrane-less organelles. While it has been recognized for some time that these organelles are of key importance for normal cellular functions, a growing number of recent observations indicate that LLPS may also play a role in disease. In particular, numerous ... Read more >>

J. Mol. Biol. (Journal of molecular biology)
[2020, :]

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Application of Marte Meo® counselling with people with behavioural variant frontotemporal dementia and their primary carers (AMEO-FTD) - a non-randomized mixed-method feasibility study.

Martin Berwig, Claudia Dinand, Ursula Becker, Margareta Halek,

Background:One of the core symptoms of behavioural variant frontotemporal dementia (bvFTD) is the early loss of social cognitive abilities, which has a deteriorating impact on everyday interaction and the quality of dyadic relationships. Marte Meo® (MM) counselling is a video-based intervention that aims to maintain or improve the quality of ... Read more >>

Pilot Feasibility Stud (Pilot and feasibility studies)
[2020, 6:32]

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Metabolomic changes associated with frontotemporal lobar degeneration syndromes

Alexander Murley, Simon Jones, Ian Coyle-Gilchrist, Lucy Bowns, Julie Wiggins, Kamen Tsvetanov, James Rowe,

Objective: Widespread metabolic changes are seen in neurodegenerative disease and could be used as biomarkers for diagnosis and disease monitoring. They may also reveal disease mechanisms that could be a target for therapy. In this study we looked for blood-based biomarkers in syndromes associated with frontotemporal lobar degeneration. Methods: Plasma ... Read more >>

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Neuropathology of Speech Network Distinguishes Bulbar From Nonbulbar Amyotrophic Lateral Sclerosis.

Sanjana Shellikeri, Julia Keith, Sandra E Black, Lorne Zinman, Yana Yunusova,

Bulbar amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative subtype affecting speech and swallowing motor functions as well as associated with the burden of cognitive deficits. The neuroanatomical underpinnings of bulbar ALS are not well understood. The aim of this study was to compare neuropathology of the speech network (SpN) ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(3):284-295]

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Predicting loss of independence and mortality in frontotemporal lobar degeneration syndromes

Alexander Murley, Matthew Rouse, Ian Coyle-Gilchrist, Simon Jones, Win Li, Julie Wiggins, Claire Lansdall, Patricia Vazquez Rodriguez, Alicia Wilcox, Karalyn Patterson, James Rowe,

Objective To test the hypothesis that in syndromes associated with frontotemporal lobar degeneration, behavioural impairment predicts loss of functional independence and motor clinical features predict mortality, irrespective of syndrome subtype. Method We used a transdiagnostic approach to survival in an epidemiological cohort, testing the association between clinical features, independence and ... Read more >>

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Plasma progranulin levels for frontotemporal dementia in clinical practice: a 10-year French experience.

Leila Sellami, Benoît Rucheton, Imen Ben Younes, Agnès Camuzat, Dario Saracino, Daisy Rinaldi, Stephane Epelbaum, Carole Azuar, Richard Levy, Sophie Auriacombe, Didier Hannequin, Jérémie Pariente, Mathieu Barbier, Claire Boutoleau-Bretonnière, Philippe Couratier, Florence Pasquier, Vincent Deramecourt, Mathilde Sauvée, Marie Sarazin, Julien Lagarde, Carole Roué-Jagot, Sylvie Forlani, Ludmila Jornea, Isabelle David, , , Eric LeGuern, Bruno Dubois, Alexis Brice, Fabienne Clot, Foudil Lamari, Isabelle Le Ber,

GRN mutations are frequent causes of familial frontotemporal degeneration. Although there is no clear consensual threshold, plasma progranulin levels represent an efficient biomarker for predicting GRN mutations when decreased. We evaluated plasma levels to determine whether it could also predict age at onset, clinical phenotype, or disease progression in 160 ... Read more >>

Neurobiol. Aging (Neurobiology of aging)
[2020, :]

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Interest of biological biomarkers in the diagnostic approach of neurocognitive disorders in the elderly.

M Lilamand, C Hourregue, C Paquet,

Alzheimer's disease (AD) is the most common cause of major neurocognitive disorders in older adults, affecting millions of individuals worldwide and leading to irreversible cognitive decline. The main neuropathological features of AD are brain amyloid deposition and neurofibrillary tangles. The biomarkers of AD are highly accurate in detecting these pathophysiological ... Read more >>

Rev. Neurol. (Paris) (Revue neurologique)
[2020, :]

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Primary Tau Pathology, Not Copathology, Correlates With Clinical Symptoms in PSP and CBD.

John L Robinson, Ning Yan, Carrie Caswell, Sharon X Xie, EunRan Suh, Vivianna M Van Deerlin, Garrett Gibbons, David J Irwin, Murray Grossman, Edward B Lee, Virginia M-Y Lee, Bruce Miller, John Q Trojanowski,

Distinct neuronal and glial tau pathologies define corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). Additional Alzheimer disease, TDP-43, and Lewy body copathologies are also common. The interplay of these pathologies with clinical symptoms remains unclear as individuals can present with corticobasal syndrome, frontotemporal dementia, PSP, or atypical Parkinsonism and ... Read more >>

J. Neuropathol. Exp. Neurol. (Journal of neuropathology and experimental neurology)
[2020, 79(3):296-304]

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Different CSF protein profiles in amyotrophic lateral sclerosis and frontotemporal dementia with C9orf72 hexanucleotide repeat expansion.

Peggy Barschke, Patrick Oeckl, Petra Steinacker, Mhd Rami Al Shweiki, Jochen H Weishaupt, G Bernhard Landwehrmeyer, Sarah Anderl-Straub, Patrick Weydt, Janine Diehl-Schmid, Adrian Danek, Johannes Kornhuber, Matthias L Schroeter, Johannes Prudlo, Holger Jahn, Klaus Fassbender, Martin Lauer, Emma Louise van der Ende, John Cornelis van Swieten, Alexander E Volk, Albert C Ludolph, Markus Otto, ,

OBJECTIVES:The hexanucleotide repeat expansion in the C9orf72 gene is the most common mutation associated with amyotrophic lateral sclerosis (C9-ALS) and frontotemporal dementia (C9-FTD). Until now, it is unknown which factors define whether C9orf72 mutation carriers develop ALS or FTD. Our aim was to identify protein biomarker candidates in the cerebrospinal ... Read more >>

J. Neurol. Neurosurg. Psychiatry (Journal of neurology, neurosurgery, and psychiatry)
[2020, :]

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Genetic architecture of neurodegenerative dementias.

Jordi Clarimon, Sonia Moreno-Grau, Laura Cervera-Carles, Oriol Dols-Icardo, Pascual Sánchez-Juan, Agustín Ruiz,

Molecular genetics has been an invaluable tool to help understand the molecular basis of neurodegenerative dementias. In this review, we provide an overview of the genetic architecture underlying some of the most prevalent causes of dementia, including Alzheimer's dementia, frontotemporal lobar degeneration, Lewy body dementia, and prion diseases. We also ... Read more >>

Neuropharmacology (Neuropharmacology)
[2020, 168:108014]

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