Abstract
Primary progressive aphasias (PPAs) are a group of neurodegenerative diseases presenting with insidious and relentless language impairment. Three main PPA variants have been described: the non-fluent/agrammatic variant (nfvPPA), the semantic variant (svPPA), and the logopenic variant (lvPPA). At the time of diagnosis, patients and their families’ main question pertains to prognosis and evolution, but very few data exist to support clinicians’ claims. The objective of this study was to review the current literature on the longitudinal changes in cognition, behaviours, and functional abilities in the three main PPA variants. A comprehensive review was undertaken via a search on PUBMED and EMBASE. Two authors independently reviewed a total of 65 full-text records for eligibility. A total of 14 group studies and one meta-analysis were included. Among these, eight studies included all three PPA variants. Eight studies were prospective, and the follow-up duration was between one and five years. Overall, svPPA patients showed more behavioural disturbances both at baseline and over the course of the disease. Patients with lvPPA showed a worse cognitive decline, especially in episodic memory, and faster progression to dementia. Finally, patients with nfvPPA showed the most significant losses in language production and functional abilities. Data regarding the prodromal and last stages of PPA are still missing and studies with a longer follow-up observation period are needed.
Highlights
Primary progressive aphasias (PPA) are a group of neurodegenerative diseases that present with an insidious, progressive, and isolated impairment in language
In addition to confirming general tendencies for the evolution of PPA, our study highlights differences in the progression of the three variants with svPPA, showing more behavioural disturbances, nfvPPA progressing towards more language and functional deficits, and lvPPA
Displaying a worse decline in global cognition, especially memory. These findings are relevant to prioritizing the clinical care offered to PPA patients and their caregivers by highlighting the challenges they are most likely to face
Summary
Primary progressive aphasias (PPA) are a group of neurodegenerative diseases that present with an insidious, progressive, and isolated impairment in language. Published diagnostic criteria for progressive non-fluent aphasia and semantic dementia [4], and in 2004, a third type of PPA was described—the logopenic variant primary progressive aphasia [5]. Diagnostic criteria for three main variants of PPA have been identified by Gorno-Tempini et al (2011) [6]. We used the classification from Gorno-Tempini et al (2011) as a framework for this study, but it is noteworthy that other clinical diagnoses and mixed cases exist even if not in the scope of this review (e.g., primary progressive apraxia of speech [7])
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