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Background and Objectives: The Baylor Profound Mental Status Examination (BPMSE) was developed to assess cognitive function in the profound stage of dementia. The Clinical Dementia Rating (CDR) scale has been widely used in measuring functional performance in dementia. We aimed to determine whether cognitive function is related to overall functional impairment in profound dementia. Methods: We selected 864 patients with probable Alzheimer disease (AD) and 25 patients with possible dementia with Lewy Bodies (DLB) cases with profound dementia by Mini-Mental Status Examination or/and clinical global impression. We used BPMSE to measure cognitive function and the CDR sum-of-boxes (CDR-SB) score to determine overall functional status. We used Spearman rank order correlation to examine the univariate association between CDR-SB and BPMSE in the 2 diagnostic groups separately and multivariable regression analysis to investigate whether BPMSE remained associated with functional status after adjustment for age, sex, education, and APOE ε4 genotype. We expected to see an inverse correlation between BPMSE and CDR-SB scores based on the directionality of the rating scale scoring. Results: In both AD and DLB, total BPMSE scores had a significant inverse correlation with CDR-SB scores (AD: r = -0.453, p < 0.001; DLB: r = -0.489, p = 0.013). It is of interest that in DLB, the "attention" domain of BPMSE had the strongest association with CDR-SB (r = -0.700, p < 0.001) compared with other domains. The multivariable regression models showed that higher BPMSE scores (i.e., better cognitive function) remained significantly correlated with lower CDR-SB scores (i.e., better global function) in AD (CDR-SB: ß = -0.340, p < 0.001), but the regression coefficient for BPMSE did not reach significance in the DLB model (CDR-SB: ß = -0.298, p = 0.174). Discussion: In patients with AD and DLB who enter the profound dementia stage, cognitive function is associated with the severity of functional impairment. The lack of significance for DLB in multivariable regression could be due to small sample size because the correlation magnitude is similar to that in AD.
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INTRODUCTION: Previous research has identified that people with cerebellar ataxia (CA) showed impaired reward-related decision-making in the Iowa Gambling Task (IGT). To investigate the mechanisms underlying this impairment, we examined CA participants' combination of performance in the IGT, which predominantly tests reward seeking, and the modified IGT (mIGT), which mainly assesses punishment avoidance. METHODS: Fifty participants with CA and one hundred controls completed the IGT and mIGT. Task performance in each of the five twenty-trial blocks was compared between groups and the learning rates were assessed with simple linear regressions. Each participant's IGT score and mIGT score were compared. RESULTS: CA participants performed worse than controls in both the IGT and the mIGT, especially in the last block (IGT: -0.24 ± 10.05 vs. 3.88 ± 10.31, p = 0.041; mIGT: 2.72 ± 7.62 vs. 8.65 ± 8.64, p < 0.001). In contrast to the controls, those with CA did not significantly improve their scores over time in either task. Controls performed better in the mIGT than the IGT, while CA participants' scores in the two tasks showed no significant difference. IGT and mIGT performance did not correlate with ataxia severity or depressive symptoms. CONCLUSION: Individuals with CA showed impaired performance in both the IGT and mIGT, which indicates disruption in both short-term reward seeking and short-term punishment avoidance. Therefore, these results suggest that reduced sensitivity to long-term consequences drives the risky decision-making in CA.