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Primary progressive aphasia: a clinical approach

Marshall, CR; Hardy, CJD; Volkmer, A; Russell, LL; Bond, RL; Fletcher, PD; Clark, CN; Mummery, CJ; Schott, JM; Rossor, MN; et al. Marshall, CR; Hardy, CJD; Volkmer, A; Russell, LL; Bond, RL; Fletcher, PD; Clark, CN; Mummery, CJ; Schott, JM; Rossor, MN; Fox, NC; Crutch, SJ; Rohrer, JD; Warren, JD (2018) Primary progressive aphasia: a clinical approach. Journal of Neurology, 265 (6). pp. 1474-1490. ISSN 0340-5354 https://doi.org/10.1007/s00415-018-8762-6
SGUL Authors: Clark, Camilla Neegaard

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Abstract

The primary progressive aphasias are a heterogeneous group of focal ‘language-led’ dementias that pose substantial challenges for diagnosis and management. Here we present a clinical approach to the progressive aphasias, based on our experience of these disorders and directed at non-specialists. We first outline a framework for assessing language, tailored to the common presentations of progressive aphasia. We then consider the defining features of the canonical progressive nonfluent, semantic and logopenic aphasic syndromes, including ‘clinical pearls’ that we have found diagnostically useful and neuroanatomical and other key associations of each syndrome. We review potential diagnostic pitfalls and problematic presentations not well captured by conventional classifications and propose a diagnostic ‘roadmap’. After outlining principles of management, we conclude with a prospect for future progress in these diseases, emphasising generic information processing deficits and novel pathophysiological biomarkers.

Item Type: Article
Additional Information: © The Author(s) 2018. This article is an open access publication Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
Keywords: 1103 Clinical Sciences, 1109 Neurosciences, Neurology & Neurosurgery
SGUL Research Institute / Research Centre: Academic Structure > Molecular and Clinical Sciences Research Institute (MCS)
Journal or Publication Title: Journal of Neurology
ISSN: 0340-5354
Language: en
Dates:
DateEvent
June 2018Published
1 February 2018Published Online
19 January 2018Accepted
Publisher License: Creative Commons: Attribution 4.0
Projects:
Project IDFunderFunder ID
AS-PG-16-007Alzheimer's SocietyUNSPECIFIED
ES/L001810/1Economic and Social Research Councilhttp://dx.doi.org/10.13039/501100000269
EP/M006093/1Engineering and Physical Sciences Research Councilhttp://dx.doi.org/10.13039/501100000266
200783Wellcome Trusthttp://dx.doi.org/10.13039/100004440
091673/Z/10/ZWellcome Trusthttp://dx.doi.org/10.13039/100004440
URI: https://openaccess.sgul.ac.uk/id/eprint/112253
Publisher's version: https://doi.org/10.1007/s00415-018-8762-6

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