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Background: Parkinson's disease psychosis (PDPsy) is associated with increased nursing home placement and mortality and is closely linked with cognitive dysfunction. Objective: Assess the clinical and cognitive features associated with PDPsy in patients without dementia. Methods: We prospectively recruited people with Parkinson's disease (PwP) without dementia for a 3-year, longitudinal study at an outpatient movement disorders clinic. Participants completed annual visits involving assessment of motor and non-motor symptoms including neuropsychological testing. PDPsy was defined as the recurring presence of visual illusions, sense of presence, hallucinations, or delusions for at least 1 month. Using generalized estimating equations, we conducted two sets of analyses to separately assess the clinical and the cognitive predictors of PDPsy. Results: We enrolled 105 participants. At baseline, mean age was 67.8 (SD = 8.0), median disease duration was 4.9 years (IQR: 3.4–7.7), and mean MoCA was 24.8 (SD = 2.3). Prevalence of PDPsy increased over 3 years from 31% (n = 32) to 39% (n = 26). Forty-five participants (43%) experienced PDPsy. Visual illusions were most common (70%, n = 84), followed by hallucinations (58.3%, n = 70). In multivariate analysis, of the clinical variables, only depressive symptoms [OR 1.09, 95% CI: (1.03, 1.16), p = 0.004] increased the odds of PDPsy; of the cognitive variables, only Trail Making Test B-A scores [OR 1.43, 95% CI: (1.06, 1.93), p = 0.018] significantly increased the odds of PDPsy. Conclusions: In PwP without dementia, depressive symptoms were associated with increased risk of PDPsy. Executive/attentional dysfunction was also associated with PDPsy and may mark the transition from isolated minor hallucinations to more complex psychotic symptoms. [ABSTRACT FROM AUTHOR]

Background: Palliative care practices, including communication about patient-centered goals of care and advance care planning (ACP), have the potential to enhance care throughout the course of Huntington's disease (HD) and related disorders. The goal of our project was to develop a pilot program that integrates primary palliative care practices with interdisciplinary care for HD. Objectives: (1) To train HD team members to facilitate goals of care and ACP conversations at all stages of HD; (2) To create materials for care planning in HD focused on patient-centered goals of care and health-related quality of life; and (3) To modify clinic workflow to include goals of care and ACP discussions. Methods: We defined planning domains to expand care planning beyond end-of-life concerns. We created a patient and family guide to advance care planning in HD. We conducted VitalTalk communications training with the HD team. We modified the interdisciplinary clinic workflow to include ACP and developed an EMR template for documentation. Results: After communication training, more team members felt well prepared to discuss serious news (12.5% to 50%) and manage difficult conversations (25% to 62.5%). The proportion of clinic visits including advance care planning discussions increased from 12.5% to 30.6% during the pilot phase. Conclusions: Provision of primary palliative care for HD in an interdisciplinary clinic is feasible. Integration of palliative care practices into HD specialty care requires additional training and modification of clinic operations. [ABSTRACT FROM AUTHOR]

Abstract Background Age at onset of Huntington's disease (HD) is correlated with the size of the abnormal CAG repeat expansion in the HD gene; however, several studies have indicated that other genetic factors also contribute to the variability in HD age at onset. To identify modifier genes, we recently reported a whole-genome scan in a sample of 629 affected sibling pairs from 295 pedigrees, in which six genomic regions provided suggestive evidence for quantitative trait loci (QTL), modifying age at onset in HD. Methods In order to test the replication of this finding, eighteen microsatellite markers, three from each of the six genomic regions, were genotyped in 102 newly recruited sibling pairs from 69 pedigrees, and data were analyzed, using a multipoint linkage variance component method, in the follow-up sample and the combined sample of 352 pedigrees with 753 sibling pairs. Results Suggestive evidence for linkage at 6q23-24 in the follow-up sample (LOD = 1.87, p = 0.002) increased to genome-wide significance for linkage in the combined sample (LOD = 4.05, p = 0.00001), while suggestive evidence for linkage was observed at 18q22, in both the follow-up sample (LOD = 0.79, p = 0.03) and the combined sample (LOD = 1.78, p = 0.002). Epistatic analysis indicated that there is no interaction between 6q23-24 and other loci. Conclusion In this replication study, linkage for modifier of age at onset in HD was confirmed at 6q23-24. Evidence for linkage was also found at 18q22. The demonstration of statistically significant linkage to a potential modifier locus opens the path to location cloning of a gene capable of altering HD pathogenesis, which could provide a validated target for therapeutic development in the human patient.

Objectives: Huntington’s disease (HD) is a neurodegenerative disorder that produces a bias toward risky, reward-driven decisions in situations where the outcomes of decisions are uncertain and must be discovered. However, it is unclear whether HD patients show similar biases in decision-making when learning demands are minimized and prospective risks and outcomes are known explicitly. We investigated how risk decision-making strategies and adjustments are altered in HD patients when reward contingencies are explicit. Methods: HD (N=18) and healthy control (HC; N=17) participants completed a risk-taking task in which they made a series of independent choices between a low-risk/low reward and high-risk/high reward risk options. Results: Computational modeling showed that compared to HC, who showed a clear preference for low-risk compared to high-risk decisions, the HD group valued high-risks more than low-risk decisions, especially when high-risks were rewarded. The strategy analysis indicated that when high-risk options were rewarded, HC adopted a conservative risk strategy on the next trial by preferring the low-risk option (i.e., they counted their blessings and then played the surer bet). In contrast, following a rewarded high-risk choice, HD patients showed a clear preference for repeating the high-risk choice. Conclusions: These results indicate a pattern of high-risk/high-reward decision bias in HD that persists when outcomes and risks are certain. The allure of high-risk/high-reward decisions in situations of risk certainty and uncertainty expands our insight into the dynamic decision-making deficits that create considerable clinical burden in HD. (JINS, 2016, 22, 426–435) [ABSTRACT FROM AUTHOR]