Results 41 to 50 of about 98,352 (316)

The neural correlates and clinical characteristics of psychosis in the frontotemporal dementia continuum and the C9orf72 expansion

NeuroImage: Clinical, 2017
Objective: This present study aims to address the gap in the literature regarding the severity and underlying neural correlates of psychotic symptoms in frontotemporal dementia with and without the C9orf72 gene expansion. Methods: Fifty-six patients with
Emma M Devenney, Ramon Landin-Romero, Muireann Irish, Michael Hornberger, Eneida Mioshi, Glenda M. Halliday, Matthew C. Kiernan, John R. Hodges   +7 more
doaj   +1 more source

Neural Substrates of Semantic Prospection – Evidence from the Dementias [PDF]

, 2016
The ability to envisage personally relevant events at a future time point represents an incredibly sophisticated cognitive endeavor and one that appears to be intimately linked to episodic memory integrity.
Abraham, Addis, Addis, Addis, Andelman, Andersson, Andersson, Ashburner, Botzung, Claire O’Callaghan, Cooper, Dermody, Duval, D’Argembeau, Frisch, Greenberg, Hassabis, Hornberger, Irish, Irish, Irish, Irish, Irish, Irish, Irish, Irish, Irish, Irish, John R. Hodges, Karantoulis, Karas, Klein, Klein, Klein, Kwan, Manning, McKhann, Mechelli, Michael Hornberger, Mioshi, Mioshi, Morris, Muireann Irish, Mullally, Mullally, Nadene Dermody, Nadine Eyre, Nichols, Okuda, Olivier Piguet, Piolino, Race, Race, Rascovsky, Reitan, Rey, Rosen, Rueckert, Savage, Schacter, Schacter, Schacter, Schmidt, Smith, Smith, Squire, Strauss, Suddendorf, Szpunar, Szpunar, Wechsler, Westmacott, Zhang   +72 more
core   +2 more sources

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

Altered Time Awareness in Dementia

Frontiers in Neurology, 2020
Our awareness of time, specifically of longer intervals spanning hours, days, months, and years, is critical for ensuring our sense of self-continuity.
Maï-Carmen Requena-Komuro, Charles R. Marshall, Charles R. Marshall, Rebecca L. Bond, Lucy L. Russell, Caroline Greaves, Katrina M. Moore, Jennifer L. Agustus, Elia Benhamou, Harri Sivasathiaseelan, Chris J. D. Hardy, Jonathan D. Rohrer, Jason D. Warren   +12 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

Broad clinical phenotypes associated with TAR-DNA binding protein (TARDBP) mutations in amyotrophic lateral sclerosis [PDF]

, 2010
The finding of TDP-43 as a major component of ubiquitinated protein inclusions in amyotrophic lateral sclerosis (ALS) has led to the identification of 30 mutations in the transactive response-DNA binding protein (TARDBP) gene, encoding TDP-43.
A Yokoseki, BA Hosler, C Lomen-Hoerth, C Vance, Christopher J. McDermott, D Neary, DR Rosen, E Buratti, E Buratti, E Kabashi, Emily F. Goodall, F Gros-Louis, GM Ringholz, H Daoud, Hannah C. Hollinger, I Gijselinck, IF Wang, IR Mackenzie, J Kirby, J Sreedharan, J. Robin Highley, Janine Kirby, JJ Kew, Judith A. Hartley, Karen E. Morrison, L Benajiba, L Corrado, M Morita, M Neumann, MJ Strong, MJ Winton, NJ Rutherford, P Kuhnlein, PA Mercado, Pamela J. Shaw, Paul G. Ince, PM Andersen, R Bo Del, R Lemmens, Rachel Hibberd, RJ Guerreiro, Rudo Masanzu, SH Ou, Stephen B. Wharton, VM Deerlin Van, William Smith, YM Ayala   +46 more
core   +1 more source

An Out‐of‐Place Etiology: Recognizing FMR1 Premutation in the Memory Clinic

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT The FMR1 gene premutation (55–200 CGG repeats) is usually associated with a wide range of symptoms and phenotypes within the Fragile X‐tremor/ataxia syndrome (FXTAS), but may also manifest as predominant or isolated cognitive decline. We describe three male patients referred for progressive cognitive impairment and behavioral changes. Standard
Guido Greco, Caterina Motta, Enrica Marchionni, Maria Rosaria D'Apice, Carlangelo Carrese, Giuseppe Novelli, Alessandro Martorana, Chiara Giuseppina Bonomi   +7 more
wiley   +1 more source

Atypical parkinsonism: An Update. [PDF]

, 2013
Purpose of review: This update discusses novel aspects on genetics, diagnosis, and treatments of atypical parkinsonism published over the past 2 years. Recent findings: A genome-wide association study identified new genetic risk factors for progressive ...
Hoeglinger, GU, Stamelou, M
core   +1 more source

Variably Protease‐Sensitive Prionopathy: Two New Cases With Motor Neuron‐Dementia Syndrome

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT We describe two patients with variably protease‐sensitive prionopathy (VPSPr) who developed progressive upper motor neuron symptoms, insomnia, behavioral and cognitive decline, compatible with primary lateral sclerosis associated with frontotemporal dementia (FTD).
María Elena Erro, María Victoria Zelaya, Hasier Eraña, Javier Sánchez Ruiz de Gordoa, Fermín García‐Amigot, Anika Simonovska‐Serra, María Cristina Caballero, Isidre Ferrer, Ellen Gelpi, Ivonne Jericó, Joaquín Castilla   +10 more
wiley   +1 more source

The exocyst subunit EXOC2 regulates the toxicity of expanded GGGGCC repeats in C9ORF72-ALS/FTD

Cell Reports
Summary: GGGGCC (G4C2) repeat expansion in C9ORF72 is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). How this genetic mutation leads to neurodegeneration remains largely unknown.
Dilara O. Halim, Gopinath Krishnan, Evan P. Hass, Soojin Lee, Mamta Verma, Sandra Almeida, Yuanzheng Gu, Deborah Y. Kwon, Thomas G. Fazzio, Fen-Biao Gao   +9 more
doaj   +1 more source