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Current Neurology and Neuroscience Reports
Published in: Current Neurology and Neuroscience Reports 3/2021

Open Access 01-03-2021 | Frontotemporal Dementia | Behavior (H.S. Kirshner, Section Editor)

Primary Progressive Aphasia: Toward a Pathophysiological Synthesis

Authors: Justina Ruksenaite, Anna Volkmer, Jessica Jiang, Jeremy CS Johnson, Charles R Marshall, Jason D Warren, Chris JD Hardy

Published in: Current Neurology and Neuroscience Reports | Issue 3/2021

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Abstract

Purpose of Review

The term primary progressive aphasia (PPA) refers to a diverse group of dementias that present with prominent and early problems with speech and language. They present considerable challenges to clinicians and researchers.

Recent Findings

Here, we review critical issues around diagnosis of the three major PPA variants (semantic variant PPA, nonfluent/agrammatic variant PPA, logopenic variant PPA), as well as considering ‘fragmentary’ syndromes. We next consider issues around assessing disease stage, before discussing physiological phenotyping of proteinopathies across the PPA spectrum. We also review evidence for core central auditory impairments in PPA, outline critical challenges associated with treatment, discuss pathophysiological features of each major PPA variant, and conclude with thoughts on key challenges that remain to be addressed.

Summary

New findings elucidating the pathophysiology of PPA represent a major step forward in our understanding of these diseases, with implications for diagnosis, care, management, and therapies.
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Metadata
Title
Primary Progressive Aphasia: Toward a Pathophysiological Synthesis
Authors
Justina Ruksenaite
Anna Volkmer
Jessica Jiang
Jeremy CS Johnson
Charles R Marshall
Jason D Warren
Chris JD Hardy
Publication date
01-03-2021
Publisher
Springer US
Published in
Current Neurology and Neuroscience Reports / Issue 3/2021
Print ISSN: 1528-4042
Electronic ISSN: 1534-6293
DOI
https://doi.org/10.1007/s11910-021-01097-z

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