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Presymptomatic and early pathological features of MAPT-associated frontotemporal lobar degeneration
Early pathological features of frontotemporal lobar degeneration (FTLD) due to MAPT pathogenic variants (FTLD-MAPT) are understudied, since...
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Increase in wasteosomes (corpora amylacea) in frontotemporal lobar degeneration with specific detection of tau, TDP-43 and FUS pathology
Wasteosomes (or corpora amylacea) are polyglucosan bodies that appear in the human brain with aging and in some neurodegenerative diseases, and have...
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Association of cortical and subcortical microstructure with disease severity: impact on cognitive decline and language impairments in frontotemporal lobar degeneration
BackgroundCortical and subcortical microstructural modifications are critical to understanding the pathogenic changes in frontotemporal lobar...
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Plasma biomarkers increase diagnostic confidence in patients with Alzheimer’s disease or frontotemporal lobar degeneration
BackgroundThe recent development of techniques to assess plasma biomarkers has changed the way the research community envisions the future of...
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Classification accuracy of blood-based and neurophysiological markers in the differential diagnosis of Alzheimer’s disease and frontotemporal lobar degeneration
BackgroundIn the last decade, non-invasive blood-based and neurophysiological biomarkers have shown great potential for the discrimination of several...
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YKL-40 changes are not detected in post-mortem brain of patients with Alzheimer’s disease and frontotemporal lobar degeneration
BackgroundYKL-40 (Chitinase 3-like I) is increased in CSF of Alzheimer’s disease (AD) and frontotemporal lobar degeneration (FTLD) patients and is...
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Intracortical diffusion tensor imaging signature of microstructural changes in frontotemporal lobar degeneration
BackgroundFrontotemporal lobar degeneration (FTLD) is a neuropathological construct with multiple clinical presentations, including the behavioural...
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Lack of a protective effect of the Tmem106b “protective SNP” in the Grn knockout mouse model for frontotemporal lobar degeneration
Genetic variants in TMEM106B are a common risk factor for frontotemporal lobar degeneration and the most important modifier of disease risk in...
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White matter hyperintensities in autopsy-confirmed frontotemporal lobar degeneration and Alzheimer’s disease
BackgroundWe aimed to systematically describe the burden and distribution of white matter hyperintensities (WMH) and investigate correlations with...
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Multimarker synaptic protein cerebrospinal fluid panels reflect TDP-43 pathology and cognitive performance in a pathological cohort of frontotemporal lobar degeneration
BackgroundSynapse degeneration is an early event in pathological frontotemporal lobar degeneration (FTLD). Consequently, a surrogate marker of...
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Clusters of co-abundant proteins in the brain cortex associated with fronto-temporal lobar degeneration
BackgroundFrontotemporal lobar degeneration (FTLD) is characterized pathologically by neuronal and glial inclusions of hyperphosphorylated tau or by...
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Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and...
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Frontotemporal lobar degeneration proteinopathies have disparate microscopic patterns of white and grey matter pathology
Frontotemporal lobar degeneration proteinopathies with tau inclusions (FTLD-Tau) or TDP-43 inclusions (FTLD-TDP) are associated with clinically...
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Frontotemporal Lobar Degeneration TDP-43-Immunoreactive Pathological Subtypes: Clinical and Mechanistic Significance
Frontotemporal lobar degeneration with TPD-43-immunoreactive pathology (FTLD-TDP) is subclassified based on the type and cortical laminar...
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Neuroimaging in Frontotemporal Lobar Degeneration: Research and Clinical Utility
Frontotemporal lobar dementia (FTLD) is a clinically and pathologically complex disease. Advances in neuroimaging techniques have provided a...
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Diffuse argyrophilic grain disease with TDP-43 proteinopathy and neuronal intermediate filament inclusion disease: FTLD with mixed tau, TDP-43 and FUS pathologies
Frontotemporal lobar degeneration (FTLD) is a group of disorders characterized by degeneration of the frontal and temporal lobes, leading to...
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Evidence of cerebellar TDP-43 loss of function in FTLD-TDP
Frontotemporal lobar degeneration with TDP-43 pathology (FTLD-TDP) is a neurodegenerative disease primarily affecting the frontal and/or temporal...
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Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration
BackgroundProgranulin loss-of-function mutations are linked to frontotemporal lobar degeneration with TDP-43 positive inclusions (FTLD-TDP- Pgrn )....
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Fluid Biomarkers of Frontotemporal Lobar Degeneration
A timely diagnosis of frontotemporal degeneration (FTD) is frequently challenging due to the heterogeneous symptomatology and poor...
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Patients with sporadic FTLD exhibit similar increases in lysosomal proteins and storage material as patients with FTD due to GRN mutations
Loss of function progranulin ( GRN ) mutations are a major autosomal dominant cause of frontotemporal dementia (FTD). Patients with FTD due to GRN ...
