Phonological Processing in Primary Progressive Aphasia

Abstract

Individuals with primary progressive aphasia (PPA) show selective breakdown in regions within the proposed dorsal (articulatory-phonological) and ventral (lexical-semantic) pathways involved in language processing. Phonological STM impairment, which has been attributed to selective damage to dorsal pathway structures, is considered to be a distinctive feature of the logopenic variant of PPA. By contrast, phonological abilities are considered to be relatively spared in the semantic variant and are largely unexplored in the nonfluent/agrammatic variant. Comprehensive assessment of phonological ability in the three variants of PPA has not been undertaken. We investigated phonological processing skills in a group of participants with PPA as well as healthy controls, with the goal of identifying whether patterns of performance support the dorsal versus ventral functional-anatomical framework and to discern whether phonological ability differs among PPA subtypes. We also explored the neural bases of phonological performance using voxel-based morphometry. Phonological performance was impaired in patients with damage to dorsal pathway structures (nonfluent/agrammatic and logopenic variants), with logopenic participants demonstrating particular difficulty on tasks involving nonwords. Binary logistic regression revealed that select phonological tasks predicted diagnostic group membership in the less fluent variants of PPA with a high degree of accuracy, particularly in conjunction with a motor speech measure. Brain-behavior correlations indicated a significant association between the integrity of gray matter in frontal and temporoparietal regions of the left hemisphere and phonological skill. Findings confirm the critical role of dorsal stream structures in phonological processing and demonstrate unique patterns of impaired phonological processing in logopenic and nonfluent/agrammatic variants of PPA.

Figure 1

Phonology scores in each PPA variant on tasks using nonword (NW) and word stimuli. Error bars represent standard errors. (Blend = Blending; Repl = Replacement)

Figure 2

Reading and spelling performance in PPA patients and healthy controls on word and nonword (NW) stimuli. Error bars represent standard errors

Figure 3

Lexicality effect sizes for written language and phonology tasks. Error bars represent standard errors.

Figure 4

Results of voxel-based morphometry (VBM; N=12 semantic variant; 12 logopenic variant; 12 nonfluent/agrammatic variant; 13 healthy controls) analysis examining relation between phonology score and gray matter volumes (p<0.001corrected for multiple comparisons based on cluster size; age, sex, total intracranial volume, motor speech score included as covariates)

Figure 5

Results of voxel-based morphometry (VBM) analysis examining relation between a) Reading nonword score and b) Spelling nonword score and gray matter volumes (p<0.001 corrected for multiple comparisons based on cluster size; age, sex, total intracranial volume included as covariates)