Results 61 to 70 of about 14,742 (206)

Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy:effects of genetic, demographic and neuropathological variables [PDF]

, 2016
Factors associated with survival were studied in 84 neuropathologically documented cases of the pre-senile dementia frontotemporal dementia lobar degeneration (FTLD) with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy ...
Armstrong, Richard A., Richard A. Armstrong   +1 more
core   +1 more source

Clinical Characteristics of C9ORF72-Linked Frontotemporal Lobar Degeneration

Dementia and Geriatric Cognitive Disorders Extra, 2013
Background: The most common genetic cause of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) has been linked to a hexanucleotide repeat expansion in the C9ORF72 gene.
Anna-Lotta Kaivorinne, Michaela K. Bode, Liisa Paavola, Hannu Tuominen, Mika Kallio, Alan E. Renton, Bryan J. Traynor, Virpi Moilanen, Anne M. Remes   +8 more
doaj   +1 more source

A 57‐Year‐Old Male With Behavioral Variant Frontotemporal Dementia and MATR3 and NOS3 Mutations

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT This report presents a case of behavioral variant frontotemporal dementia caused by mutations in the MATR3 and NOS3 genes, aiming to analyze its clinical manifestations and genetic characteristics. For a case presenting with personality changes and gait abnormalities as the initial symptoms, this study conducted a comprehensive analysis of its
Feifei Lin, Saie Huang
wiley   +1 more source

Protein Disulfide Isomerase Disassembles TDP‐43/G3BP1 Condensates and Antagonizes TDP‐43 Pathological Aggregates

Advanced Science, EarlyView.
Cytoplasmic aggregation of TDP‐43 is a common pathological feature in amyotrophic lateral sclerosis, frontotemporal lobar degeneration, and Alzheimer's disease with TDP‐43 pathology. This study reports that wild‐type PDI slows down phase separation of TDP‐43 through direct interaction with TDP‐43.
Jia‐Qi Liu, Hao Liu, Yu‐Xuan Sun, Yuying Li, Xiangyi Liu, Li‐Qiang Wang, Zhaofei Yang, Qi Fu, Xiaojiao Xu, Jie Chen, Yingshuang Zhang, Jun Zhou, Weidong Le, Mengchao Cui, Yi Liang   +14 more
wiley   +1 more source

TDP‐43 Aggregation: The Healthy‐Toxic Balance of the Prion‐Like Domain

Advanced Science, EarlyView.
TDP‐43 function relies on a delicate balance between reversible phase‐separated states and irreversible aggregation. Under physiological conditions, TDP‐43 forms dynamic droplets and oligomers that support normal cellular functions. In pathological contexts, this balance shifts toward aberrant aggregation, leading to toxic species.
Luca Zangrando, Emanuele Buratti, Francesca Paron   +2 more
wiley   +1 more source

GRN mutation in a patient with a behavioral variant of frontotemporal lobar degeneration (bvFTD)

, 2017
The clinical spectrum of frontotemporal lobar degeneration (FTLD) is characterized by personality changes, language impairment, and executive function deficits.
Sylwia Walczysková, Šárka Hilscherová, Jiří Konrád, Jaroslav Kotlas, Pavel Ressner, Věnceslava Svobodová   +5 more
core   +1 more source

Recent biomarker approaches in the diagnosis of frontotemporal lobar degeneration [PDF]

, 2012
Frontotemporal lobar degeneration (FTLD) is a heterogeneous group of syndromes with different symptoms. Frontotemporal lobar degeneration is mostly used as a clinical umbrella term for different diseases. In some clinical subtypes of the FTLD spectrum, a
Steinacker, Petra, Neumann, Manuela, Feneberg, Emily, Otto, Markus, Lehnert, Stefan   +4 more
core   +1 more source

Exploration of novel biomarkers in frontotemporal lobar degeneration [PDF]

, 2023
Frontotemporal lobar degeneration is a neurodegenerative disorder characterised by progressive deterioration of frontal and anterior temporal lobes of the brain. It can be divided into a number of distinct clinical syndromes including semantic variant of
Hwang, Yun Tae
core  

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

A Reproducible Deep-Learning-Based Computer-Aided Diagnosis Tool for Frontotemporal Dementia Using MONAI and Clinica Frameworks

Life, 2022
Despite Artificial Intelligence (AI) being a leading technology in biomedical research, real-life implementation of AI-based Computer-Aided Diagnosis (CAD) tools into the clinical setting is still remote due to unstandardized practices during development.
Andrea Termine, Carlo Fabrizio, Carlo Caltagirone, Laura Petrosini, on behalf of the Frontotemporal Lobar Degeneration Neuroimaging Initiative   +4 more
doaj   +1 more source