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Temporal Variant Frontotemporal Dementia Is Associated with Globular Glial Tauopathy

Clark, CN; Lashley, T; Mahoney, CJ; Warren, JD; Revesz, T; Rohrer, JD (2015) Temporal Variant Frontotemporal Dementia Is Associated with Globular Glial Tauopathy. Cognitive And Behavioral Neurology, 28 (2). pp. 92-97. ISSN 1543-3633
SGUL Authors: Clark, Camilla Neegaard

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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.

Item Type: Article
Additional Information: Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial License 4.0 International (CCBY-NC 4.0), where it is permissible to download, share, remix, transform, and build up the work provided it is properly cited. The work cannot be used commercially.
Keywords: 11 Medical and Health Sciences, 17 Psychology and Cognitive Sciences, Neurology & Neurosurgery, Psychiatry
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Journal or Publication Title: Cognitive And Behavioral Neurology
ISSN: 1543-3633
Language: en
June 2015Published
31 December 2014Accepted
Publisher License: Creative Commons: Attribution-Noncommercial 4.0
Project IDFunderFunder ID
091673/Z/10/ZWellcome Trust
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