Abstract
BackgroundThe evidence about the language performance profile of multiple system atrophy (MSA) is limited, but its definition may lead to a more comprehensive characterization of the disorder and contribute to clarify the involvement of the basal ganglia in language abilities.ObjectiveThe objectives of the study were: (1) to evaluate the reliability of the Screening for Aphasia in NeuroDegeneration (SAND) in MSA patients; (2) compare the linguistic profiles among MSA and Parkinson’s disease (PD) patients and healthy controls (HC), and (3) assess relationships between language impairment and cognitive status and MSA motor subtypes.Methods and resultsForty patients with a diagnosis of MSA, 22 HC and 17 patients with PD were enrolled in the present study. By excluding the writing task that showed a poor acceptability, we showed that the MSA-tailored SAND Global Score is an acceptable, consistent and reliable tool to screen language disturbances in MSA. MSA patients performed worse than HC, but not than PD, in MSA-tailored SAND Global Score, repetition, reading and semantic association tasks. We did not find significant differences between MSA phenotypes. MSA patients with mild cognitive impairment-multiple domain presented worse language performances as compared to MSA patients with normal cognition and mild cognitive impairment-single domain.ConclusionThe MSA-tailored SAND Global Score is a consistent and reliable tool to screen language disturbances in MSA. Language disturbances characterize MSA patients irrespective of disease phenotype, and parallel the decline of global cognitive functions.
Highlights
Multiple system atrophy (MSA) is a rapidly progressive α-synucleinopathy, characterized by different combinations of progressive parkinsonism, cerebellar ataxia, autonomic failure and corticospinal impairment
Parkinson’s disease (PD) patients as well as 22 healthy controls (HC), matched for age, education, cognitive state assessed by Mini-Mental State Evaluation (MMSE) and disease duration
As for the other cognitive tests, moderate correlation was present with measures of global cognition as the MMSE and the Montreal Cognitive Assessment (MOCA), but no correlation was shown with memory test and apathy and depression scores
Summary
Multiple system atrophy (MSA) is a rapidly progressive α-synucleinopathy, characterized by different combinations of progressive parkinsonism, cerebellar ataxia, autonomic failure and corticospinal impairment. The poor performance on language production tasks might be primarily the consequence of speech disorders, such as dysarthria, which is a common clinical feature of atypical parkinsonian syndromes and is related to basal ganglia pathology. Objective The objectives of the study were: (1) to evaluate the reliability of the Screening for Aphasia in NeuroDegeneration (SAND) in MSA patients; (2) compare the linguistic profiles among MSA and Parkinson’s disease (PD) patients and healthy controls (HC), and (3) assess relationships between language impairment and cognitive status and MSA motor subtypes. By excluding the writing task that showed a poor acceptability, we showed that the MSA-tailored SAND Global Score is an acceptable, consistent and reliable tool to screen language disturbances in MSA. Conclusion The MSA-tailored SAND Global Score is a consistent and reliable tool to screen language disturbances in MSA. Language disturbances characterize MSA patients irrespective of disease phenotype, and parallel the decline of global cognitive functions
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