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Primary progressive aphasia: ReADing the clinical GRANularity

Chokesuwattanaskul, A; Marshall, CR; Van Harskamp, N; Houlden, H; Rohrer, J; Hardy, CJD; Warren, JD; (2022) Primary progressive aphasia: ReADing the clinical GRANularity. Practical Neurology 10.1136/practneurol-2022-003460. (In press). Green open access

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Abstract

Primary progressive aphasia remains a diagnostic challenge despite (or even because of) the increasing availability of ancillary tests and biomarkers. We present a 67-year-old man with apparently sporadic logopenic aphasia and positive Alzheimer biomarkers who was subsequently found also to have a pathogenic mutation in the progranulin gene. This was signalled by early atypical features (mild expressive agrammatism and behavioural change, rapid clinical deterioration) around the core logopenic aphasia syndrome. Each of the canonical progressive aphasia syndromes has a 'halo' of less typical variants that may herald alternative or additional pathologies. The accurate diagnosis of primary progressive aphasia depends on careful clinical analysis to direct investigations appropriately.

Type: Article
Title: Primary progressive aphasia: ReADing the clinical GRANularity
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1136/practneurol-2022-003460
Publisher version: http://dx.doi.org/10.1136/practneurol-2022-003460
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. // The Dementia Research Centre is supported by Alzheimer's Research UK, Brain Research UK, and the Wolfson Foundation. This work was supported by the Alzheimer’s Society, Alzheimer’s Research UK and the National Institute for Health Research University College London Hospitals Biomedical Research Centre. CRM is supported by a grant from Bart’s Charity. CJDH is supported by a RNID-Dunhill Medical Trust Pauline Ashley Fellowship ((PA23) JDW receives grant support from Alzheimer’s Research UK, the Alzheimer’s Society and the National Brain Appeal (Frontotemporal Dementia Research Studentship in Memory of David Blechner). JDR is supported by the Miriam Marks Brain Research UK Senior Fellowship and has received funding from an MRC Clinician Scientist Fellowship (MR/M008525/1) and the NIHR Rare Disease Translational Research Collaboration (BRC149/NS/MH). For the purpose of open access, the author has applied a Creative Commons Attribution (CC BY) licence to any Author Accepted Manuscript version arising.
UCL classification: 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 > Neurodegenerative Diseases
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
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 > Department of Neuromuscular Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10151749
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