title: Temporal Variant Frontotemporal Dementia is Associated with Globular Glial Tauopathy.
abstract: Frontotemporal dementia (FTD) is a clinically and pathologically heterogeneous neurodegenerative disorder associated with atrophy of the frontal and temporal lobes. Most patients with focal temporal lobe atrophy present with either the semantic dementia subtype of FTD or the behavioral variant subtype. For patients with temporal variant FTD, the most common cause found on post-mortem examination has been a TDP-43 (transactive response DNA-binding protein 43 kDa) proteinopathy, but tauopathies have also been described, including Pick's disease and mutations in the microtubule-associated protein tau (MAPT) gene. We report the clinical and imaging features of 2 patients with temporal variant FTD associated with a rare frontotemporal lobar degeneration pathology known as globular glial tauopathy. The pathologic diagnosis of globular glial tauopathy should be considered in patients with temporal variant FTD, particularly those who have atypical semantic dementia or an atypical parkinsonian syndrome in association with the right temporal variant.
authors.affiliation: *Dementia Research Centre †Queen Square Brain Bank for Neurological Disorders, University College London, Institute of Neurology, London, United Kingdom.
authors.forename: Camilla N
authors.initials: CN
authors.lastname: Clark
authors.forename: Tammaryn
authors.initials: T
authors.lastname: Lashley
authors.forename: Colin J
authors.initials: CJ
authors.lastname: Mahoney
authors.forename: Jason D
authors.initials: JD
authors.lastname: Warren
authors.forename: Tamas
authors.initials: T
authors.lastname: Revesz
authors.forename: Jonathan D
authors.initials: JD
authors.lastname: Rohrer
mesh_terms: D001284:Atrophy / Q000473:pathology; D001344:Autopsy; D005260:Female; D005625:Frontal Lobe / Q000378:metabolism / Q000473:pathology*; D057180:Frontotemporal Dementia / Q000150:complications / Q000378:metabolism* / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D024801:Tauopathies / Q000150:complications / Q000378:metabolism* / Q000473:pathology*; D013702:Temporal Lobe / Q000378:metabolism / Q000473:pathology; D016875:tau Proteins / Q000378:metabolism
chemical_list: D016875:tau Proteins