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Abstract Background Huntington's disease (HD) is a heritable degenerative brain disease caused by a mutation in the huntingtin gene with excessive repeats of the base triplet cytosine–adenine–guanine (CAG), which codes for the aminoacid glutamine. HD is associated with a broad spectrum of neurocognitive dysfunction, including deficits in social cognition. The appreciation of fairness rules and reciprocity has not been studied in HD. Based on theoretical considerations suggesting that brain regions known to be affected from HD are involved in economic decision‐making, the present study sought to examine HD patients' performance in two neuroeconomic games. Methods Twenty‐nine manifest HD mutation carriers (20 males, nine females) performed an Ultimatum Game (UG) and a Dictator Game (DG) where third‐party punishment of observed unfairness was required. In addition, patients were tested for neurocognition and the ability to understand other people's mental states (“theory of mind”). For comparison, a clinical control group of 30 patients with chronic schizophrenia, and 30 unaffected healthy controls matched for age and verbal intelligence took part in the study. Results Patients with HD had some appreciation of fairness rules, as they tended to reject unfair offers in the UG similar to controls. However, unlike the other two groups, individuals with HD did not punish observed unfairness from a third‐party perspective. This lack of “altruistic punishment” was associated with deficits in executive functioning including working memory, inhibitory control and cognitive flexibility, and to a lesser degree with poor “theory of mind.” Conclusions HD seems to be associated with impairments in understanding of more complex rules of social exchange. Aside from deficits in executive functioning, this behavior could, in part, be linked to an inability to experience third‐party punishment as rewarding.

Abstract Objective To investigate cognitive flexibility in premanifest and manifest Huntington's disease (HD). Background HD is an autosomal dominant neurodegenerative disease characterized by motor, cognitive, and behavioral abnormalities with typical motor symptoms. In this study, we wanted to assess decision making in premanifest (pre‐HD) and manifest HD patients. Methods A total of 77 non‐demented subjects including 29 pre‐HD, 22 manifest HD patients, and 26 healthy controls (HC) were included. We stratified the pre‐HD group based on their estimated years to disease onset into a far (FAR, n = 13) and a near (NEAR, n = 16) group. Furthermore, participants performed the Montreal cognitive assessment battery (MoCA), the trail making task part A and B (TMT A, TMT B), the Symbol digit modalities test (SDMT), and the beads task. Results In the beads task, HD patients gathered less information than all other groups (all p‐values

Abstract Background Motor phenotypes in Huntington's disease vary manifold. Phenotype classification is essential to adapt treatment. The aim of this study was to classify a dystonic subtype closer. Methods A total of 7,512 manifest ENROLL‐HD participants were subdivided into mainly choreatic (N = 606), dystonic (N = 402), and hypokinetic‐rigid (N = 369) subjects. Cognitive (verbal fluency, symbol digit, stroop color, trail making, Mini‐Mental State Examination), functional (total functional capacity, Independence Scale), and psychiatric (problem behaviors assessment, Hospital Anxiety and Depression Scale) performance was evaluated at baseline visit. Results Symptoms onset for dystonic were similar to hypokinetic‐rigid, but earlier compared to choreatic subjects (p