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

LC Lin, AL Nana, M Hepker, JHL Hwang… - Acta neuropathologica …, 2019 - Springer
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 …

Novel CSF biomarkers for frontotemporal lobar degenerations

WT Hu, A Chen-Plotkin, M Grossman, SE Arnold… - Neurology, 2010 - AAN Enterprises
Objective: To identify antemortem CSF diagnostic biomarkers that can potentially distinguish
between the 2 main causes of frontotemporal lobar degeneration (FTLD), ie, FTLD with TDP …

Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease

NJ Brandmeir, F Geser, LK Kwong, E Zimmerman… - Acta …, 2008 - Springer
Abstract Recently, TDP-43, a 43 kDa nuclear TAR DNA-binding protein, was identified as
the major disease protein in frontotemporal lobar degeneration with ubiquitinated inclusions …

Absence of FUS-immunoreactive pathology in frontotemporal dementia linked to chromosome 3 (FTD-3) caused by mutation in the CHMP2B gene

IE Holm, AM Isaacs, IRA Mackenzie - Acta neuropathologica, 2009 - Springer
Frontotemporal dementia linked to chromosome 3 (FTD-3) is a very rare disease described
in a large Danish family [1] and also in an unrelated Belgian patient with familial FTD [12]. A …

TDP-43 pathology in familial frontotemporal dementia and motor neuron disease without Progranulin mutations

H Seelaar, H Jurgen Schelhaas, A Azmani, B Küsters… - Brain, 2007 - academic.oup.com
Frontotemporal dementia is accompanied by motor neuron disease (FTD+ MND) in∼ 10%
of cases. There is accumulating evidence for a clinicopathological overlap between FTD and …

A reassessment of the neuropathology of frontotemporal dementia linked to chromosome 3

IE Holm, E Englund, IRA Mackenzie… - … of Neuropathology & …, 2007 - academic.oup.com
A large Danish family has previously been reported in which autosomal dominant
frontotemporal dementia (FTD) is genetically linked to chromosome 3 (FTD-3). A mutation …

Genotype–phenotype links in frontotemporal lobar degeneration

S Van Mossevelde, S Engelborghs… - Nature Reviews …, 2018 - nature.com
Frontotemporal lobar degeneration (FTLD) represents a group of neurodegenerative brain
diseases with highly heterogeneous clinical, neuropathological and genetic characteristics …

Expansion of the classification of FTLD-TDP: distinct pathology associated with rapidly progressive frontotemporal degeneration

EB Lee, S Porta, G Michael Baer, Y Xu, ER Suh… - Acta …, 2017 - Springer
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) can typically be
categorized into one of four distinct histopathologic patterns of TDP-43 pathology, types A to …

Neuropathologic, biochemical, and molecular characterization of the frontotemporal dementias

RT Mott, DW Dickson, JQ Trojanowski… - Journal of …, 2005 - academic.oup.com
The frontotemporal dementias (FTDs) are a heterogeneous group of neurodegenerative
disorders that are characterized clinically by dementia, personality changes, language …

Familial frontotemporal dementia with ubiquitin-positive inclusions is linked to chromosome 17q21–22

SM Rosso, W Kamphorst, B de Graaf, R Willemsen… - Brain, 2001 - academic.oup.com
Hereditary frontotemporal dementia (FTD) is an autosomal dominant neurodegenerative
disorder that is associated with mutations in the tau gene and with the pathological …