Evolution of language abilities over the course of the disease in a large cohort of patients with lvPPA: More than a repetition impairment

Abstract

AbstractBackgroundInitial language impairment in lvPPA causes naming and repetition difficulties along with phonological errors (Gorno‐Tempini et al., 2011). Little is known about the evolution of other language capacities with disease progression. This study aimed at exploring the full range of language impairments throughout the course of the disease, from onset to death.MethodWe performed a retrospective chart review on a large cohort of deceased lvPPA patients (n = 38; 18 M, 19 F; Mean age at onset = 69 years old). Presence or absence of impairment in various language abilities was determined via consensus by three experienced raters at 5 time points from onset to death.ResultAll 38 patients developed anomia, repetition impairment and writing difficulties throughout disease course (Figure 1). The overall prevalence of reading difficulties was also high (Figure 1), with impairment appearing more than two years post‐onset in most cases (Figure 2). Comprehension deficits were highly prevalent, appearing mostly in the first two years post onset (Figure 2). More than 75% of patients showed grammar difficulties (Figure 1), mainly in later stages of the disease (Figure 2). Semantic deficits also appeared later (Figure 2). Finally, apraxia of speech was quite infrequent (Figure 1).ConclusionIn addition to known language deficits in lvPPA (i.e., naming, repetition, reading and writing, see Brambati et al., 2009; Faria et al., 2013; Gorno‐Tempini et al., 2011; Sepelyak et al., 2011), our results suggest early impairments in comprehension as well as late grammar difficulties. To our knowledge, this is the first study which addresses the full range of language impairments in lvPPA over the entire course of the disease. We believe this is highly relevant for patients/families to better prepare for the future, as well as therapists to design more focused interventions.