Clinical, Anatomical, and Pathological Features in the Three Variants of Primary Progressive Aphasia: A Review

Maxime Montembeault, Simona M Brambati, Maria Luisa Gorno-Tempini, Raffaella Migliaccio, Maxime Montembeault, Simona M Brambati, Maria Luisa Gorno-Tempini, Raffaella Migliaccio

Abstract

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.

Keywords: brain connectivity; language; logopenic variant; nonfluent/agrammatic variant; primary progressive aphasia; semantic variant.

Figures

Figure 1
Figure 1
Areas of significant atrophy in a group of patients with nonfluent/agrammatic variant of primary progressive aphasia (nfvPPA) and then subdivided by pathological subgroups. The main areas of atrophy are mainly in the left inferior frontal gyrus, insula and premotor areas. No significant difference was observed in the comparison between nfvPPA-tau and nfvPPA-TDP-43 patients in this study. However, greater white matter damage has been reported in nfvPPA-tau cases (69). FWE, family wise error; 4R-tau, nfvPPA patients with FTD-tau with 4 repeats pathology; PiD, nfvPPA patients with Pick's disease pathology; TDP-A, nfvPPA patients with FTD-TDP-43 depositions type A pathology; AD + CBD, nfvPPA patients with Alzheimer's disease and corticobasal degeneration pathologies; GM, gray matter; WM, white matter; L, left [from (45), Permission to reproduce have been obtained from the copyright holders of this work].
Figure 2
Figure 2
Speech production white matter tracts are shown in healthy controls, nfvPPA, lvPPA, and svPPA patients. Fractional anisotropy (FA) (A) and mean diffusivity (MD) (B) metrics were evaluated in four speech production tracts (BA44-SMAc, BA44-Putamen, SMAc-Caudate, and BA44-Caudate) and in the inferior longitudinal fasciculus (ILF). FA is a measure that quantifies the degree to which the diffusion of water molecules in WM fiber bundles is restricted to a specific direction (a higher FA value is associated with a better structural integrity). MD is a measure that quantifies the average degree of diffusion of water molecules in all directions (a lower MD value is associated with a better structural integrity). These results suggest that the white matter tracts connecting the speech production network are selectively damaged in nfvPPA. BA44, Broadmann Area 44; SMAc, Supplementary motor area complex; ILF, inferior longitudinal fasciculus. [from (46), Permission to reproduce have been obtained from the copyright holders of this work].
Figure 3
Figure 3
Left language-related white matter tracts are shown in healthy controls, nfvPPA, svPPA, and lvPPA patients. Fractional anisotropy (FA) (A) and mean diffusivity (MD) (B) metrics were evaluated in three tracts: the SLF (dorsal pathway), the ILF and UNC (ventral pathways). FA is a measure that quantifies the degree to which the diffusion of water molecules in WM fiber bundles is restricted to a specific direction (a higher FA value is associated with a better structural integrity). MD is a measure that quantifies the average degree of diffusion of water molecules in all directions (a lower MD value is associated with a better structural integrity). In nfvPPA, the main damage was observed in the dorsal pathway. In svPPA, the two ventral pathways as well as the temporal part of the dorsal pathway was damaged. In lvPPA, only the temporoparietal part of the dorsal pathway was damaged. SLF, Superior longitudinal fasciculus; ILF: inferior longitudinal fasciculus; UNC: uncinated fasciculus; FA, fractional anisotropy; MD, mean diffusivity [from (51), Permission to reproduce have been obtained from the copyright holders of this work].
Figure 4
Figure 4
(A) Areas of significant atrophy in a group of patients with semantic variant of primary progressive aphasia (svPPA) and subdivided by pathological subgroups. The main areas of atrophy are in the bilateral anterior temporal lobes, more predominantly in the left hemisphere. (B) Areas of significant atrophy in svPPA patients with FTD tau pathology, in comparison to those with TDP-43 pathology. These patients present greater atrophy of frontotemporal cortex (medial anterior temporal lobe, orbitofrontal cortex, anterior cingulate cortex), basal ganglia and connecting white matter structures. FWE, family wise error; TDP-C, svPPA patients with FTD-TDP-43 depositions type C pathology; TDP-C+ tau nos, svPPA patients with FTD-TDP-43 depositions type C and tau pathology not otherwise specified; PiD, svPPA patients with Pick's disease pathology; GGT, svPPA patients with globular glial tauopathy pathology; L, left; R, right; GM, gray matter; WM, white matter [from (45), Permission to reproduce have been obtained from the copyright holders of this work].
Figure 5
Figure 5
Comparison of areas of hypoperfusion between groups of lvPPA patients with AD pathology, lvPPA patients without AD pathology and typical AD patients. LvPPA patients present significant hypoperfusion in the left temporal-parietal junction, affecting larger portions of the temporal cortex in the lvPPA patients with AD pathology. In comparison to typical AD patients, lvPPA patients with AD pathology show more significant hypoperfusion in the left superior temporal gyrus. LPA, lvPPA; LPA-, lvPPA patients without AD pathology; LPA+, lvPPA patients with AD pathology; AD, Alzheimer's disease [from (149), Permission to reproduce have been obtained from the copyright holders of this work].

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