Results 91 to 100 of about 30,886 (249)

[¹⁸F]PI-2620 Binding Patterns in Patients with Suspected Alzheimer Disease and Frontotemporal Lobar Degeneration [PDF]

, 2023
Ganna Blazhenets, David N. Soleimani‐Meigooni, Wesley P. Thomas, Nidhi S. Mundada, Matthias Brendel, Stephanie Vento, Lawren VandeVrede, Hilary W. Heuer, Peter A. Ljubenkov, Julio C. Rojas, Miranda Chen, Alinda Amuiri, Zachary Miller, Maria Luisa Gorno‐Tempini, Bruce L. Miller, Howie Rosen, Irene Litvan, Murray Grossman, Bradley F. Boeve, Alexander Pantelyat, Maria Carmela Tartaglia, David J. Irwin, Brad C. Dickerson, Suzanne L. Baker, Adam L. Boxer, Gil D. Rabinovici, Renaud La Joie   +26 more
openalex   +1 more source

Serum neurofilament light chain is a discriminative biomarker between frontotemporal lobar degeneration and primary psychiatric disorders

Journal of Neurology, 2019
Due to the significant clinical overlap between frontotemporal lobar degeneration (FTLD) spectrum disorders and late-onset primary psychiatric disorders (PPD), diagnostic biomarkers reflecting the different underlying pathophysiologies are urgently ...
Kasper Katisko, Antti Cajanus, Olli Jääskeläinen, Aleksi Kontkanen, P. Hartikainen, V. Korhonen, S. Helisalmi, A. Haapasalo, H. Koivumaa-Honkanen, S. Herukka, A. Remes, E. Solje   +11 more
semanticscholar   +1 more source

Epilepsy in dentatorubral–pallidoluysian atrophy: A systematic review and meta‐analysis

Epilepsia, EarlyView.
Summary of key clinical and electrophysiological characteristics of DRPLA‐related epilepsy from a systematic review and meta‐analysis of 1,191 patients. DRPLA patients with epilepsy showed earlier disease onset, longer CAG repeat expansion, and a tendency toward paternal inheritance. EEG findings frequently included photoparoxysmal responses.
Toru Horinouchi, Haruka Ishibashi, Yukako Nakagami, Yoko Kobayashi Takahashi, Takato Akiba, Masaharu Miyauchi, Naohiro Yamamoto, Ryoichi Inoue, Satoshi Kodama, Takafumi Kubota, Naoto Kuroda   +10 more
wiley   +1 more source

Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity

Nature Communications, 2018
TDP-43 is associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitinated inclusions (FTD-TDP). Here, the authors identify the transcriptional elongation factor Ell as a strong modifier of TDP-43-mediated ...
Chia-Yu Chung, Amit Berson, Jason R. Kennerdell, Ashley Sartoris, Travis Unger, Sílvia Porta, Hyung-Jun Kim, Edwin R. Smith, Ali Shilatifard, Vivianna Van Deerlin, Virginia M.-Y. Lee, Alice Chen-Plotkin, Nancy M. Bonini   +12 more
doaj   +1 more source

Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degeneration

Acta Neuropathologica Communications, 2017
An intronic GGGGCC expansion in C9orf72 is the most common known cause of both frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). The repeat expansion leads to the generation of sense and antisense repeat RNA aggregates and
Sarah Mizielinska, Charlotte E. Ridler, Rubika Balendra, Annora Thoeng, Nathan S. Woodling, Friedrich A. Grässer, Vincent Plagnol, Tammaryn Lashley, Linda Partridge, Adrian M. Isaacs   +9 more
doaj   +1 more source

Association of cortical and subcortical microstructure with disease severity: impact on cognitive decline and language impairments in frontotemporal lobar degeneration

Alzheimer’s Research & Therapy, 2023
Background Cortical and subcortical microstructural modifications are critical to understanding the pathogenic changes in frontotemporal lobar degeneration (FTLD) subtypes. In this study, we investigated cortical and subcortical microstructure underlying
Wencai Ding, Peng Ren, Liye Yi, Yao Si, Fan Yang, Zhipeng Li, Hongbo Bao, Shi Yan, Xinyu Zhang, Siyang Li, Xia Liang, Lifen Yao, for the Frontotemporal Lobar Degeneration Neuroimaging Initiative   +12 more
doaj   +1 more source

Physiological, behavioral and subjective sadness reactivity in frontotemporal dementia subtypes. [PDF]

, 2019
Frontotemporal dementia (FTD), a neurodegenerative disease broadly characterized by socioemotional impairments, includes three clinical subtypes: behavioral variant FTD (bvFTD), semantic variant primary progressive aphasia (svPPA) and non-fluent variant ...
Brown, Casey L, Casey, James J, Chen, Kuan-Hua, Hua, Alice Y, Levenson, Robert W, Lwi, Sandy J, Miller, Bruce L, Rosen, Howard J   +7 more
core  

C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins [PDF]

, 2014
An expanded GGGGCC repeat in C9orf72 is the most common genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis. A fundamental question is whether toxicity is driven by the repeat RNA itself and/or by dipeptide repeat proteins ...
Cabecinha, M, Clayton, EL, Cleverley, K, Devoy, A, Dols, J, Fisher, EMC, Fratta, P, Grönke, S, Hendrich, O, Isaacs, AM, Mizielinska, S, Moens, T, Niccoli, T, Nicoll, AJ, Norona, FE, Partridge, L, Pickering-Brown, S, Pietrzyk, J, Ridler, CE, Woollacott, IOC   +19 more
core   +1 more source

Predicting loss of independence and mortality in frontotemporal lobar degeneration syndromes

Journal of Neurology Neurosurgery & Psychiatry, 2020
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 diagnostic group. Methods
A. Murley, M. Rouse, I. Coyle-Gilchrist, P. S. Jones, Win-Yin Li, J. Wiggins, C. Lansdall, Patricia Vázquez Rodríguez, A. Wilcox, K. Patterson, James B. Rowe   +10 more
semanticscholar   +1 more source

TDP‐43 proteinopathies and neurodegeneration: insights from Caenorhabditis elegans models

The FEBS Journal, EarlyView.
The manuscript explores structural and functional features of TDP‐43 and its worm homologue, TDP‐1, highlighting conserved and divergent structural and functional features. Using genetically engineered C. elegans models, key pathological features of TDP‐43 proteinopathies—including aggregation, neurodegeneration, and motor deficits—are recapitulated ...
Ghulam Jeelani Pir, Joerg Buddenkotte, Majid Ali Alam, Ahmed Own, Randall J. Eck, Brian C. Kraemer, Eckhard Mandelkow, Martin Steinhoff   +7 more
wiley   +1 more source