Peripheral Innate Immune Activation Correlates With Disease Severity in GRN Haploinsufficiency. [PDF]
Objective: To investigate associations between peripheral innate immune activation and frontotemporal lobar degeneration (FTLD) in progranulin gene (GRN) haploinsufficiency. Methods: In this cross-sectional study, ELISA was used to measure six markers of
Allen, Isabel Elaine +16 more
core +2 more sources
Exploring the role of brain DNA methylomic signatures on gene expression dynamics of frontotemporal lobar degeneration [PDF]
Katerine Fodder +11 more
openalex +1 more source
Expanding the Phenotype of Frontotemporal Lobar Degeneration With FUS-Positive Pathology (FTLD-FUS) [PDF]
Karina Chornenka +6 more
openalex +1 more source
A Case of Frontotemporal Lobar Degeneration with Progressive Dysarthria
We investigated the evolution of the neurological and neuropsychological characteristics in a right-handed woman who was 53-years-old at the onset and who showed personality changes and behavioral disorders accompanied by progressive dysarthria.
Nami Ihori +3 more
doaj +1 more source
Neuroanatomical profiles of personality change in frontotemporal lobar degeneration [PDF]
Colin Mahoney +4 more
openalex +1 more source
Molecular Pathways Bridging Frontotemporal Lobar Degeneration and Psychiatric Disorders
The overlap of symptoms between neurodegenerative and psychiatric diseases has been reported. Neuropsychiatric alterations are commonly observed in dementia, especially in the behavioral variant of frontotemporal dementia (bvFTD), which is the most ...
Roberta eZanardini +3 more
doaj +1 more source
Loss-of-function mutations affecting the lysosomal protein progranulin are a leading cause of frontotemporal dementia. Progranulin mutations cause abnormalities in lysosomal lipid processing, particularly of sphingolipids, major components of neural cell
Nicholas R. Boyle +12 more
doaj +1 more source
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies [PDF]
Accumulation of hyperphosphorylated, ubiquitinated and N-terminally truncated TAR DNA-binding protein (TDP-43) is the pathological hallmark lesion in most familial and sporadic forms of FTLD-U and ALS, which can be subsumed as TDP-43 proteinopathies.
Flatley, Andrew +10 more
core
Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy: effects of genetic, demographic and neuropathological variables [PDF]
Richard A. Armstrong
openalex +1 more source
Frontotemporal Lobar Degeneration
Frontotemporal lobar degeneration (FTLD) comprises diseases with a very diverging spectrum in regards to clinical presentation, genetics, and neuropathology. In 1892 Arnold Pick published the case of 71-year old male with progressive symptoms of aphasia, apathy, and dementia (Pick 1892).
openaire +3 more sources

