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Primary progressive aphasia: conceptual evolution and challenges

Authors Leyton C, Ballard K

Received 19 December 2015

Accepted for publication 26 February 2016

Published 29 April 2016 Volume 2016:5 Pages 9—18


Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 3

Editor who approved publication: Professor SH Annabel Chen

Cristian E Leyton, Kirrie J Ballard
Faculty of Health Sciences, The University of Sydney, Lidcombe, NSW, Australia

Abstract: Since the modern description of primary progressive aphasia (PPA) more than 30 years ago, the interest in neurodegenerative conditions that selectively target the language network has grown exponentially. Fueled by advances in neuroimaging and biomarkers, progress in the field has brought new insights into clinical categorization, neural correlates of language, and pathological mechanisms of progression in PPA. Of relevance, the inception of logopenic progressive aphasia as an atypical presentation of Alzheimer's disease and the formalization of the international diagnostic criteria and classification of PPA represent milestones in the field. Paradoxically, those advances have also brought controversy and challenges. The application of the current classification in cases with mixed or very mild language deficits is still challenging, while the accurate pathological prediction at the individual level remains elusive. In addition, it is more evident now that nonlanguage deficits, including other cognitive and motor deficits, can appear early on and potentially assist in the differential diagnosis. From a historical perspective, this review addresses the conceptual evolution of PPA and the contribution that clinical refinements, cognitive neuropsychology, and pathology have made to the field.

Keywords: primary progressive aphasia, nonfluent variant, logopenic variant, frontotemporal lobar degeneration, frontotemporal dementia, semantic dementia, Alzheimer's disease

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