UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

A case of TDP-43 type C pathology presenting as nonfluent variant primary progressive aphasia

Adams-Carr, KL; Bocchetta, M; Neason, M; Holton, JL; Lashley, T; Warren, JD; Rohrer, JD; (2020) A case of TDP-43 type C pathology presenting as nonfluent variant primary progressive aphasia. Neurocase , 26 (1) pp. 1-6. 10.1080/13554794.2019.1690665. Green open access

[thumbnail of Rohrer_A case of TDP-43 type C pathology presenting as nonfluent variant primary progressive aphasia_AAM.pdf]
Preview
Text
Rohrer_A case of TDP-43 type C pathology presenting as nonfluent variant primary progressive aphasia_AAM.pdf - Accepted Version

Download (1MB) | Preview

Abstract

We report a case of rapidly progressive nonfluent variant PPA (nfvPPA), age at onset 77 years old and disease duration 3.3 years, who came to post mortem and was found to have TDP-43 type C pathology, an unusual finding for nfvPPA. All prior TDP-43 type C cases from the UCL FTD cohort (n=25) had a semantic variant PPA (svPPA) phenotype, with all having a younger age at onset and longer disease duration than the nfvPPA case. Volumetric analysis of MRI from the nfvPPA case, twelve of the svPPA cases and ten age-matched controls was performed. Whilst left frontal and insular volumes were lower in the nfvPPA case compared with svPPA, cortical and medial temporal lobe volumes were lower (particularly on the right) in the svPPA group compared with the nfvPPA patient. Such anatomical involvement is likely to be consistent with the presence of a nonfluent aphasia (left frontal lobe and insula), and only mild semantic deficit early in the illness (left but not right temporal lobe). Such unique cases add to the heterogeneity of the FTD spectrum.

Type: Article
Title: A case of TDP-43 type C pathology presenting as nonfluent variant primary progressive aphasia
Open access status: An open access version is available from UCL Discovery
DOI: 10.1080/13554794.2019.1690665
Publisher version: https://doi.org/10.1080/13554794.2019.1690665
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Frontotemporal dementia, nfvPPA, svPPA, TDP-43, primary progressive aphasia
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10087524
Downloads since deposit
88Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item