The language profile in multiple system atrophy: an exploratory study

Abstract

Background The 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. 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. Methods and results Forty 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. 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.

A Patient with Multiple System Atrophy-Parkinsonian Type Presenting with Progressive Micrographia

“…6 Cuoco et al compared the linguistic profiles among MSA and PD patients and HC using the “Screening for Aphasia in NeuroDegeneration” (SAND) questionnaire. 7 In conclusion, they found that MSA patients performed worse in total MSA-tailored SAND Global Score, as compared to HC ( p < 0.01) 7 and the writing subscore was lowest in the MSA group. However, the authors removed the subscores of the writing task from analyses for the reason that the motor dysfunction of MSA could affect the writing task, which requires involvement of the motor circuit in addition to the language networks.…”

“…However, the authors removed the subscores of the writing task from analyses for the reason that the motor dysfunction of MSA could affect the writing task, which requires involvement of the motor circuit in addition to the language networks. 7 We think that the subtype of PM, which requires the disturbance of a widespread neural network, 3,4 surely constitutes a more intriguing motor phenomenon other than the classical micrographia and other motor symptoms. The improvement in PM with levodopa in our MSA subject was not notable, supporting the involvement of neurotransmitter systems other than dopaminergic as hypothesized in PD subjects.…”

“…However, the authors removed the subscores of the writing task from analyses for the reason that the motor dysfunction of MSA could affect the writing task, which requires involvement of the motor circuit in addition to the language networks. 7…”

Sentence completion in progressive supranuclear palsy following transcranial direct current stimulation

“…According to Lazzeri et al 19 , dementia is present in up to 15% of MSA patients, generally manifesting in the later disease stages. Besides word fluency, language disorders affecting naming, repetition, and reading as well as simple sentence structure have been described in MSA 20 .…”