Clinical, anatomical and pathological features in the three variants of primary progressive aphasia : a review
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Abstract(s)
Primary progressive aphasias (PPA) are neurodegenerative diseases clinically
characterized by an early and relatively isolated language impairment. Three main
clinical variants, namely the nonfluent/agrammatic variant (nfvPPA), the semantic
variant (svPPA), and the logopenic variant (lvPPA) have been described, each with
specific linguistic/cognitive deficits, corresponding anatomical and most probable
pathological features. Since the discovery and the development of diagnostic criteria for
the PPA variants by the experts in the field, significant progress has been made in the
understanding of these diseases. This review aims to provide an overview of the literature
on each of the PPA variant in terms of their clinical, anatomical and pathological features,
with a specific focus on recent findings. In terms of clinical advancements, recent
studies have allowed a better characterization and differentiation of PPA patients based
on both their linguistic and non-linguistic profiles. In terms of neuroimaging, techniques
such as diffusion imaging and resting-state fMRI have allowed a deeper understanding
of the impact of PPA on structural and functional connectivity alterations beyond the
well-defined pattern of regional gray matter atrophy. Finally, in terms of pathology,
despite significant advances, clinico-pathological correspondence in PPA remains far
from absolute. Nonetheless, the improved characterization of PPA has the potential to
have a positive impact on the management of patients. Improved reliability of diagnoses
and the development of reliable in vivo biomarkers for underlying neuropathology will
also be increasingly important in the future as trials for etiology-specific treatments
become available.
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