Abstract

BackgroundProgressive Supranuclear Palsy (PSP) patients present language disturbances in tasks like naming, repetition, reading, word comprehension and semantic association compared to Parkinson’s disease (PD) and healthy controls (HC).ObjectiveIn the present study we sought to validate a Screening for Aphasia in NeuroDegeneration (SAND) battery version specifically tailored on PSP patients and to describe language impairment in relation to PSP disease phenotype and cognitive status.Methods and resultsFifty-one PSP [23 with Richardson’s syndrome (PSP-RS), 10 with predominant parkinsonism (PSP-P) and 18 with the other variant syndromes of PSP (vPSP)], 28 PD and 30 HC were enrolled in the present study. By excluding the tasks with poor acceptability (i.e., writing and picture description tasks) and increasing the items related to the remaining tasks, we showed that the PSP-tailored SAND Global Score is an acceptable, consistent and reliable tool to screen language disturbances in PSP. However, we failed to detect major differences in language involvement according to disease phenotype. Differently, we showed that patients with dementia present worse language performances.ConclusionsTaking into account specific disease features, the combination of the SAND subscores included in the PSP-tailored SAND better represents language abilities in PSP. Furthermore, we showed that language disturbances feature PSP patients irrespective of disease phenotype, but parallels the deterioration of the global cognitive function.

Highlights

  • Progressive Supranuclear Palsy (PSP) is a rare, rapidly progressive neurodegenerative disease characterized by postural instability and supranuclear vertical gaze palsy as well as by cognitive and behavioral symptoms [1]

  • By excluding the tasks with poor acceptability and increasing the items related to the remaining tasks, we showed that the PSP-tailored Screening for Aphasia in NeuroDegeneration (SAND) Global Score is an acceptable, consistent and reliable tool to screen language disturbances in PSP

  • We showed that language disturbances feature PSP patients irrespective of disease phenotype, but parallels the deterioration of the global cognitive function

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Summary

Introduction

Progressive Supranuclear Palsy (PSP) is a rare, rapidly progressive neurodegenerative disease characterized by postural instability and supranuclear vertical gaze palsy as well as by cognitive and behavioral symptoms [1]. According to the clinical diagnostic criteria proposed by the Movement Disorder Society (MDS)[2], language impairment is part of the complex spectrum of disturbances affecting patients with PSP. Evidence suggests that a wide spectrum of language deficits characterize the remaining PSP phenotypes, including Richardson’s syndrome (PSP-RS). Burrell et al reported that patients with PSP-RS present specific language deficits to those affected by Primary Progressive Aphasia (PPA)[3]. There is scant of evidence on the language profile of PSP patients diagnosed according to MDS clinical criteria [4]. Progressive Supranuclear Palsy (PSP) patients present language disturbances in tasks like naming, repetition, reading, word comprehension and semantic association compared to Parkinson’s disease (PD) and healthy controls (HC)

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