Abstract

Anomia is common in Primary Progressive Aphasia (PPA), and there is considerable evidence that semantic problems (as opposed to impaired access to output word phonology) exist in many PPA individuals irrespective of their strict subtype, including a loss of representations from semantic memory, which is typical for people with the semantic variant of PPA. In this manuscript we present a straightforward novel clinical algorithm that quantifies this degree of semantic storage impairment. We sought to produce an algorithm by employing tasks that would measure key elements of semantic storage loss: a) whether an unrecalled name could be retrieved with cues; b) if performance for items was consistent across tasks; and c) the degree to which a participant’s performance was related to general severity of cognitive impairment rather than semantic loss. More specifically, these tasks were given to 28 individuals with PPA (12 participants had a clinical diagnosis of atypical Alzheimer’s Disease with the logopenic variant of PPA; the remaining 16 participants received a clinical diagnosis of Frontotemporal dementia (11 were classified as the non-fluent variant of PPA and five were the semantic variant of PPA). Scores from these tasks produced a single omnibus semantic memory storage loss score (SSL score) for each person that ranged from 0.0 to 1.0, with scores closer to 0 more indicative of semantic storage loss. Indeed, supporting the hypothesis that our scores measure the degree of semantic storage loss, we found participants with the semantic variant of PPA had the lowest scores, and SSL scores could predict the degree of hypometabolism in the anterior temporal lobe; even when only people with the logopenic variant of PPA were examined. Thus, these scores show promise quantitating the degree of a person’s semantic representation loss.

Highlights

  • Individuals with acquired brain damage to the left hemisphere often demonstrate the inability to name pictures

  • Twelve participants were classified as having a clinical diagnosis of logopenic variant of PPA (lvPPA)

  • 11 were classified as non-fluent variant of PPA (nfvPPA) according to the core criteria of Gorno-Tempini [27]

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Summary

Introduction

Individuals with acquired brain damage to the left hemisphere often demonstrate the inability to name pictures (anomia). In many individuals with stroke-induced Broca’s aphasia (or non-fluent Primary Progressive Aphasia), the problem lies not with knowledge of concepts, but arises only at the output lexical level, wherein the sound forms of words are contacted [1, 2]. In another set of individuals with anomia from stroke or neurodegenerative disease, the locus of impairment producing anomia lies at the level of semantic cognition [3, 4]. This latter network is highly reminiscent of earlier concepts regarding “semantic access disorders” [8, 12,13,14,15]

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