Primary Progressive Aphasia: Toward a Pathophysiological Synthesis.

Affiliation

Ruksenaite J(1), Volkmer A(2), Jiang J(1), Johnson JC(1), Marshall CR(3), Warren JD(#)(1), Hardy CJ(#)(4).
Author information:
(1)Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, 8 - 11 Queen Square, London, WC1N 3BG, UK.
(2)Division of Psychology and Language Sciences, University College London, London, UK.
(3)Preventive Neurology Unit, Wolfson Institute of Preventive Medicine, Queen Mary University of London, London, UK.
(4)Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, 8 - 11 Queen Square, London, WC1N 3BG, UK. [Email]
(#)Contributed equally

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. 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.