Abstract

Mounting evidence suggests that, in parallel with well-defined changes in language, primary progressive aphasia (PPA) syndromes display co-occurring social cognitive impairments. Here, we explored multidimensional profiles of carer-rated social communication using the La Trobe Communication Questionnaire (LCQ) in 11 semantic dementia (SD), 12 logopenic progressive aphasia (LPA) and 9 progressive non-fluent aphasia (PNFA) cases and contrasted their performance with 19 Alzheimer’s disease (AD) cases, 26 behavioural variant frontotemporal dementia (bvFTD) cases and 31 healthy older controls. Relative to the controls, the majority of patient groups displayed significant overall social communication difficulties, with common and unique profiles of impairment evident on the LCQ subscales. Correlation analyses revealed a differential impact of social communication disturbances on functional outcomes in patient and carer well-being, most pronounced for SD and bvFTD. Finally, voxel-based morphometry analyses based on a structural brain MRI pointed to the degradation of a distributed brain network in mediating social communication dysfunction in dementia. Our findings suggest that social communication difficulties are an important feature of PPA, with significant implications for patient function and carer well-being. The origins of these changes are likely to be multifactorial, reflecting the breakdown of fronto-thalamic brain circuits specialised in the integration of complex information.

Highlights

  • Introduction published maps and institutional affilPrimary progressive aphasia (PPA) refers to a collection of diverse neurodegenerative clinical syndromes characterised by progressive deterioration of language and speech functions

  • A total of 108 participants took part in this study, of which 12 patients with a clinical diagnosis of logopenic progressive aphasia (LPA), 11 patients diagnosed with semantic dementia (SD; 4 cases with right-sided SD) and 9 patients diagnosed with progressive non-fluent aphasia (PNFA) [1] were contrasted with 19 clinically probable Alzheimer’s disease (AD) [35] and 26 clinically probable behavioural-variant frontotemporal dementia [36] cases

  • Significant group differences were evident in the ACE-III total scores (F(5,100) = 15.701, p < 0.0001, ηp 2 = 0.440), with LPA, SD, behavioural variant frontotemporal dementia (bvFTD) and AD patient groups showing deficits in overall cognitive function relative to controls

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Summary

Introduction

Primary progressive aphasia (PPA) refers to a collection of diverse neurodegenerative clinical syndromes characterised by progressive deterioration of language and speech functions. Current diagnostic criteria recognise three PPA variants based on distinct profiles of linguistic changes, distribution of brain atrophy and underlying neuropathology [1]. These variants are semantic variant PPA (referred to here as semantic dementia (SD)), nonfluent/agrammatic variant PPA (or progressive non-fluent aphasia (PNFA)) and logopenic variant PPA (or logopenic progressive aphasia (LPA)), each of which is characterised by differential patterns of language and motor speech dysfunction. Impoverished language is a prototypical feature of LPA, manifesting in slowing of spontaneous speech, phonological errors and paraphasias, sentence repetition and word-finding difficuliations

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