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.

No abstract

The objective of the present study was to describe gait parameters of progressive supranuclear palsy (PSP) phenotypes at early stage verifying the ability of gait analysis in discriminating between disease phenotypes and between the other variant syndromes of PSP (vPSP) and Parkinson's disease (PD). Nineteen PSP (10 PSP-Richardson's syndrome, five PSP-parkinsonism, and four PSP-progressive gait freezing) and nine PD patients performed gait analysis in single and dual tasks. Although phenotypes showed similar demographic and clinical variables, Richardson's syndrome presented worse cognitive functions. Gait analysis demonstrated worse parameters in Richardson's syndrome compared with the vPSP. The overall diagnostic accuracy of the statistical model during dual task was almost 90%. The correlation analysis showed a significant relationship between gait parameters and visuo-spatial, praxic, and attention abilities in PSP-Richardson's syndrome only. vPSP presented worse gait parameters than PD. Richardson's syndrome presents greater gait dynamic instability since the earliest stages than other phenotypes. Computerized gait analysis can differentiate between PSP phenotypes and between vPSP and PD.

Introduction: The aim of this study is to investigate the impact of orthostatic hypotension (OH) on cognitive functions in patients with multiple system atrophy (MSA) followed over time.Methods: Thirty-two MSA patients were enrolled and underwent a comprehensive neuropsychological battery; at baseline (T0) 15 out of 32 patients presented OH, assessed by means of orthostatic standing test. All patients underwent a follow-up (T1) evaluation 12 months after baseline. Thirteen out of 32 patients also underwent a second follow-up (T2) evaluation at 24 months. Changes over time on different neuropsychological tasks were compared between patients with and without OH by means of Mann-Whitney's U-test. Moreover, clinical categories of normal cognition, mild cognitive impairment, and dementia were determined, and changes at T1 and T2 in global cognitive status were compared between patients with and without OH.Results: At T0, patients with OH had better performance on words/non-words repetition task (p = 0.02) compared to patients without OH. Compared to patients without OH, patients with OH performed worse on semantic association task (p < 0.01) at T1 and on Stroop test-error effect (p = 0.04) at T2. The percentage of patients with worsened cognitive status at T1 was higher among patients with OH than among patients without OH (93 vs. 59%, p = 0.03). OH (β = −4.67, p = 0.01), education (β = 0.45, p = 0.02), age (β = 0.19, p = 0.03), and Montreal Cognitive Assessment battery (MOCA) score at T0 (β = −0.26, p = 0.04) were significant predictors of global cognitive status worsening at T1.Discussion: We found that global cognitive status worsened at 1-year follow-up in 93% of patients with OH, and OH, along with age, education, and MOCA score, predicted cognitive worsening over time. To clarify the relationship between OH and cognitive dysfunction in MSA, we suggest the use of clinical categories of normal cognition, mild cognitive impairment, and dementia in further longitudinal studies on MSA patients with and without OH.

No abstract