Abstract
Huntington’s disease (HD) is an inherited neurodegenerative disorder characterized by involuntary, jerky movements, incoordination, behavioral changes and subtle executive and cognitive impairment starting before motor symptoms. Our study aimed to assess the risky decision-making process in premanifest (pre) HD subjects, by means Game of Dice Task (GDT). As dependent variables, several GDT outcomes have been taken into consideration. We recruited 30 subjects (15 females) with preHD (i.e., Diagnosis Confidence Level < 4; Total Motor Score < 10), and 21 age, gender and education matched neurologically normal subjects (11 females). GDT is a computer-guided task where subjects are invited to watch the digits on which to bet and to evaluate the related potential risk to win or loss. Our results showed that decision and feedback times were longer in preHD than in neurologically normal group in both disadvantageous and advantageous choices. PreHD subjects provided a greater number of “safe” strategies, taken with longer decision-making time than neurologically normal subjects, showing a reduced propensity to risk. Such behavior, characterized by increased slowness in acting and providing answers, might contribute to delineate a behavioral and cognitive profile in preHD.
Highlights
Huntington’s disease (HD) is a rare, neurogenetic disorder caused by a CAG expanded mutation causing involuntary movements, incoordination, behavioral changes and cognitive decline
No statistically significant differences between two groups have been observed with respect to the number of risky choices
The aim of our study was to assess decision-making under objective risk in Premanifest HD (preHD) stage using a Game of Dice Task (GDT), in an attempt to identify typical potential predictors of executive impairment that anticipate other cognitive and motor signs in HD
Summary
Huntington’s disease (HD) is a rare, neurogenetic disorder caused by a CAG expanded mutation causing involuntary movements, incoordination, behavioral changes and cognitive decline. Cognitive abnormal functions may anticipate motor symptoms and may contribute to behavioral alterations (Stout et al, 2011; Bates et al, 2015). Functional alterations depend on neurodysfunctional processes affecting striatal and cortical neurons, e.g., neocortex, hippocampus, and thalamus (Fennema-Notestine et al, 2004; Ciarmiello et al, 2006; Wolf and Klöppel, 2013) with frontal-subcortical brain circuit abnormalities (Rosas et al, 2002; Thieben et al, 2002; Kassubek et al, 2004; Douaud et al, 2006) contributing to affect executive functions and decision-making since early stages of HD. Premanifest (pre) HD subjects performed as efficiently as control subjects, showing no decision-making impairment under objective risk, while manifest HD patients showed a wider executive impairment involving several abilities
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