Results 41 to 50 of about 23,647 (218)

Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach

Brain Sciences, 2023
Frontotemporal lobar degeneration (FTLD) belongs to a heterogeneous group of highly complex neurodegenerative diseases and represents the second cause of presenile dementia in individuals under 65.
Genaro Gabriel Ortiz, Javier Ramírez-Jirano, Raul L. Arizaga, Daniela L. C. Delgado-Lara, Erandis D. Torres-Sánchez   +4 more
doaj   +1 more source

Progranulin in frontotemporal lobar degeneration and neuroinflammation

Journal of Neuroinflammation, 2007
Progranulin (PGRN) is a pleiotropic protein that has gained the attention of the neuroscience community with recent discoveries of mutations in the gene for PGRN that cause frontotemporal lobar degeneration (FTLD).
Hutton Michael L, Mackenzie Ian RA, Ahmed Zeshan, Dickson Dennis W   +3 more
doaj   +1 more source

Cerebrospinal Fluid Tau, p-Tau 181 and Amyloid-beta(38/40/42) in Frontotemporal Dementias and Primary Progressive Aphasias [PDF]

, 2010
Background/Aims: We determined cerebrospinal fluid (CSF) concentrations of amyloid-beta(A beta)(1-38), A beta(1-40), A beta(1-42), total tau and phospho-tau (p-tau) in order to study their differential expression in frontotemporal dementia (FTD, n = 25 ...
Bibl, Mirko, Esselmann, Hermann, Kornhuber, Johannes, Lewczuk, Piotr, Mollenhauer, Brit, Otto, Markus, Rüther, Eckart, Wiltfang, Jens, Wolf, Stefanie   +8 more
core   +1 more source

Fluid and Neuroimaging Biomarkers in Microgliopathy Colony‐Stimulating Factor‐1 Receptor‐Related Disorders

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective This study aims to identify both fluid and neuroimaging biomarkers for CSF1R‐RD that can inform the optimal timing of treatment administration to maximize therapeutic benefit, while also providing sensitive quantitative measurements to monitor disease progression.
Tomasz Chmiela, Madison Reeves, Karen Jansen‐West, Judith Dunmore, Yuping Song, Audrey Strongosky, Sunil Gandhi, Gilana Pikover, Matt Blurton‐Jones, Robert C. Spitale, Erik H. Middlebrooks, Leonard Petrucelli, Mercedes Prudencio, Zbigniew K. Wszolek   +13 more
wiley   +1 more source

Value of MRI Outcomes for Preventive and Early‐Stage Trials in Spinocerebellar Ataxias 1 and 3

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective To examine the value of MRI outcomes as endpoints for preventive and early‐stage trials of two polyglutamine spinocerebellar ataxias (SCAs). Methods A cohort of 100 participants (23 SCA1, 63 SCA3, median Scale for the Assessment and Rating of Ataxia (SARA) score = 5, 42% preataxic, and 14 gene‐negative controls) was scanned at 3T up ...
Thiago J. R. Rezende, Emilien Petit, Young Woo Park, Sophie Tezenas du Montcel, James M. Joers, Jonathan M. DuBois, H. Moore Arnold, Michal Povazan, Ipek Özdemir, Guita Banan, Romain Valabregue, Philipp Ehses, Jennifer Faber, Chiadi U. Onyike, Peter B. Barker, Jeremy D. Schmahmann, Eva‐Maria Ratai, Sub H. Subramony, Thomas H. Mareci, Khalaf O. Bushara, Henry Paulson, Thomas Klockgether, Alexandra Durr, Tetsuo Ashizawa, Christophe Lenglet, Gülin Öz, READISCA Investigators   +26 more
wiley   +1 more source

The novel MAPT mutation K298E:mechanisms of mutant tau toxicity, brain pathology and tau expression in induced fibroblast-derived neurons [PDF]

, 2013
Frontotemporal lobar degeneration (FTLD) consists of a group of neurodegenerative diseases characterized by behavioural and executive impairment, language disorders and motor dysfunction.
Calo, Laura, Goedert, Michel, Holton, Janice L., Iovino, Mariangela, Lashley, Tammaryn, Lund University., Lund University., Lund University., Muqit, Miratul M. K., Parmar, Malin,, Pfisterer, Ulrich,, Revesz, Tamas, Spillantini, Maria Grazia, Swingler, Robert J., Treacy, Rebecca   +14 more
core   +4 more sources

TBK1‐Associated Primary Lateral Sclerosis Followed by Right Temporal Variant Frontotemporal Dementia

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT We report a 58‐year‐old woman with a novel splice‐site variant in the TANK‐binding kinase 1 (TBK1:c.993–2A>C p.Ala332TyrfsTer39) who sequentially developed primary lateral sclerosis (PLS) followed by right temporal variant frontotemporal dementia (rtvFTD). Neuroimaging demonstrated right anterior temporal atrophy before cognitive symptoms, and
Tomoyasu Matsubara, Naoki Kihara, Satoko Miyatake, Koji Fujita, Konoka Tachibana, Ryosuke Miyamoto, Hiroki Yamazaki, Yusuke Osaki, Nazere Keyoumu, Yuki Kuwano, Nobutoshi Morimoto, Suzuran Saito, Eriko Koshimizu, Yoichi Otomi, Kenji Ishibashi, Masafumi Harada, Naomichi Matsumoto, Hiroyuki Morino, Yuishin Izumi   +18 more
wiley   +1 more source

A Case of Frontotemporal Dementia with Epilepsia Partialis Continua Responding to Lacosamide Treatment

Archives of Epilepsy, 2019
Seizures may occur in frontotemporal lobar degeneration syndromes as an element of a heterogeneous group of disorders, according to both clinical phenotype and neuropathology.
Yasemin DİNÇ, Gönül ÇELİK AKDAĞ
doaj   +1 more source

Cognitive and olfactory function profiles of frontotemporal lobar degeneration

Chinese Journal of Contemporary Neurology and Neurosurgery, 2021
Objective To describe the cognitive and olfactory function profiles of frontotemporal lobar degeneration (FTLD). Methods A total of 52 FTLD patients admitted to Tianjin Huanhu Hospital from July 2014 to April 2017 were enrolled.
DOU Yu⁃chao, LI Yu⁃qing, JI Yong, LI Pan, ZHOU Yu⁃ying   +4 more
doaj   +1 more source

Preferential tau aggregation in von Economo neurons and fork cells in frontotemporal lobar degeneration with specific MAPT variants. [PDF]

, 2019
Tau aggregation is a hallmark feature in a subset of patients with frontotemporal dementia (FTD). Early and selective loss of von Economo neurons (VENs) and fork cells within the frontoinsular (FI) and anterior cingulate cortices (ACC) is observed in ...
Coppola, Giovanni, Cosme, Celica G, Gan, Li, Gaus, Stephanie E, Geschwind, Daniel H, Grinberg, Lea T, Hepker, Mackenzie, Hwang, Ji-Hye Lee, Lin, Li-Chun, Miller, Bruce L, Nana, Alissa L, Rosen, Howard J, Seeley, William W, Spina, Salvatore   +13 more
core   +1 more source