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Case report of a patient with unclassified tauopathy with molecular and neuropathological features of both progressive supranuclear palsy and corticobasal degeneration
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are distinct clinicopathological subtypes of frontotemporal lobar...
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[18F]-FDopa positron emission tomography imaging in corticobasal syndrome
PurposeFirst, to investigate the patterns of [ 18 F]-FDOPA positron emission tomography imaging in corticobasal syndrome using visual and...
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Progressive Supranuclear Palsy and Corticobasal Degeneration
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are neurodegenerative tauopathies with neuronal and glial lesions composed...
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Fiber-specific micro- and macroscopic white matter alterations in progressive supranuclear palsy and corticobasal syndrome
Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) are characterized by progressive white matter (WM) alterations associated with...
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MAPT subhaplotypes in corticobasal degeneration: assessing associations with disease risk, severity of tau pathology, and clinical features
The microtubule-associated protein tau ( MAPT ) H1 haplotype is the strongest genetic risk factor for corticobasal degeneration (CBD). However, the...
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Associations of mitochondrial genomic variation with corticobasal degeneration, progressive supranuclear palsy, and neuropathological tau measures
Mitochondrial health is important in ageing and dysfunctional oxidative phosphorylation (OXPHOS) accelerates ageing and influences neurodegeneration....
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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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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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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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Grey-matter correlates of empathy in 4-Repeat Tauopathies
Loss of empathy is an early and central symptom of frontotemporal lobar degeneration spectrum diseases. We aimed to investigate the topographical...
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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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Pretectum p1 (Prosomere 1)
Structures dealt with in this chapter according to genoarchitectonical reasons no longer can be summarized under the term brainstem (Watson et al.,...
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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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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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Determinants of astrocytic pathology in stem cell models of primary tauopathies
Astrocytic tau aggregates are seen in several primary and secondary tauopathies, including progressive supranuclear palsy (PSP), corticobasal...
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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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Neuropathological changes associated with aberrant cerebrospinal fluid p-tau181 and Aβ42 in Alzheimer’s disease and other neurodegenerative diseases
Recent studies suggest that increased cerebrospinal fluid (CSF) phospho-tau is associated with brain amyloid pathology rather than the tau pathology....
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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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Tau Protein and Frontotemporal Dementias
Filamentous inclusions of tau protein are found in cases of inherited and sporadic frontotemporal dementias (FTDs). Mutations in MAPT, the tau gene,...
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Clinical and Neuroimaging Aspects of Familial Frontotemporal Lobar Degeneration Associated with MAPT and GRN Mutations
Numerous kindreds with familial frontotemporal lobar degeneration have been linked to mutations in microtubule-associated protein tau (MAPT) or...