Primary Progressive Aphasia: Toward a Pathophysiological Synthesis

• Published in: Current neurology and neuroscience reports Vol. 21; no. 3; p. 7
• Main Authors: Ruksenaite, Justina, Volkmer, Anna, Jiang, Jessica, Johnson, Jeremy CS, Marshall, Charles R, Warren, Jason D, Hardy, Chris JD
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.03.2021; Springer Nature B.V
• Subjects: Aphasia; Aphasia, Primary Progressive - diagnosis; Behavior (H.S. Kirshner; Behavior (H.S. Kirshner, Section Editor); Diagnosis; Humans; Language; Medicine; Medicine & Public Health; Neurology; Neurosciences; Phenotyping; Reviews; Section Editor; Speech; Topical Collection on Behavior; Logopenic aphasia; Semantic dementia; Progressive nonfluent aphasia; Alzheimer’s disease; Physiology; Primary progressive aphasia; Frontotemporal dementia
• ISSN: 1528-4042; 1534-6293
• DOI: 10.1007/s11910-021-01097-z

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.