Frontotemporal lobar degeneration

M Grossman, WW Seeley, AL Boxer, AE Hillis… - Nature Reviews …, 2023 - nature.com
Frontotemporal lobar degeneration (FTLD) is one of the most common causes of early-onset
dementia and presents with early social–emotional–behavioural and/or language changes …

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

IRA Mackenzie, R Rademakers… - The Lancet Neurology, 2010 - thelancet.com
Abnormal intracellular protein aggregates comprise a key characteristic in most
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …

[HTML][HTML] Age at symptom onset and death and disease duration in genetic frontotemporal dementia: an international retrospective cohort study

KM Moore, J Nicholas, M Grossman… - The Lancet …, 2020 - thelancet.com
Background Frontotemporal dementia is a heterogenous neurodegenerative disorder, with
about a third of cases being genetic. Most of this genetic component is accounted for by …

Progranulin, lysosomal regulation and neurodegenerative disease

AW Kao, A McKay, PP Singh, A Brunet… - Nature Reviews …, 2017 - nature.com
The discovery that heterozygous and homozygous mutations in the gene encoding
progranulin are causally linked to frontotemporal dementia and lysosomal storage disease …

Clinical, genetic and pathological heterogeneity of frontotemporal dementia: a review

H Seelaar, JD Rohrer, YAL Pijnenburg… - Journal of Neurology …, 2011 - jnnp.bmj.com
Frontotemporal dementia (FTD) is the second most common young-onset dementia and is
clinically characterised by progressive behavioural change, executive dysfunction and …

Neuropathology underlying clinical variability in patients with synucleinopathies

GM Halliday, JL Holton, T Revesz, DW Dickson - Acta neuropathologica, 2011 - Springer
Abnormal aggregates of the synaptic protein, α-synuclein, are the dominant pathology in
syndromes known as the synucleinopathies. The cellular aggregation of the protein occurs …

REM sleep behavior disorder: updated review of the core features, the REM sleep behavior disorder‐neurodegenerative disease association, evolving concepts …

BF Boeve - Annals of the New York Academy of Sciences, 2010 - Wiley Online Library
Rapid eye movement (REM) sleep behavior disorder (RBD) is a parasomnia manifested by
vivid, often frightening dreams associated with simple or complex motor behavior during …

[HTML][HTML] Neuropathological background of phenotypical variability in frontotemporal dementia

KA Josephs, JR Hodges, JS Snowden… - Acta …, 2011 - Springer
Frontotemporal lobar degeneration (FTLD) is the umbrella term encompassing a
heterogeneous group of pathological disorders. With recent discoveries, the FTLDs have …

Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics

JL Whitwell, SD Weigand, BF Boeve, ML Senjem… - Brain, 2012 - academic.oup.com
A major recent discovery was the identification of an expansion of a non-coding GGGGCC
hexanucleotide repeat in the C9ORF72 gene in patients with frontotemporal dementia and …

Clinical and neuroanatomical signatures of tissue pathology in frontotemporal lobar degeneration

JD Rohrer, T Lashley, JM Schott, JE Warren, S Mead… - Brain, 2011 - academic.oup.com
Relating clinical symptoms to neuroanatomical profiles of brain damage and ultimately to
tissue pathology is a key challenge in the field of neurodegenerative disease and …