scholarly journals Problems with Social Cognition and Decision-Making in Huntington’s Disease: Why Is it Important?

2021 ◽  
Vol 11 (7) ◽  
pp. 838
Author(s):  
Sarah L. Mason ◽  
Miriam Schaepers ◽  
Roger A. Barker

Huntington’s disease starts slowly and progresses over a 15–20 year period. Motor changes begin subtly, often going unnoticed by patients although they are typically visible to those close to them. At this point, it is the early non-motor problems of HD that arguably cause the most functional impairment. Approximately 65% of gene carriers will experience a reduction in their occupational level, and just under half will feel unable to manage their finances independently before a clinical diagnosis is made. Understanding what drives this impairment in activities of daily living is the key to helping people with HD to live more independently for longer, especially in early disease. Early cognitive decline is likely to play a contributory factor although few studies have looked directly at this relationship. Recently, it has been shown that along with the well documented dysexecutive syndrome seen in HD, changes in social cognition and decision-making are more common than previously thought. Furthermore, some of the early neuropathological and neurochemical changes seen in HD disrupt networks known to be involved in social functioning. In this review, we explore how HD changes the way individuals interact in a social world. Specifically, we summarise the literature on both classical and social decision-making (value-based decision-making in a social context) along with studies of theory of mind, empathy, alexithymia, and emotion recognition in HD. The literature specific to HD is discussed and supported by evidence from similar neurodegenerative disorders and healthy individuals to propose future directions and potential therapeutic avenues to be explored.

2019 ◽  
Vol 10 ◽  
Author(s):  
Giulia D’Aurizio ◽  
Simone Migliore ◽  
Giuseppe Curcio ◽  
Ferdinando Squitieri

2021 ◽  
Vol 10 (21) ◽  
pp. 5181
Author(s):  
Klaudia Plinta ◽  
Andrzej Plewka ◽  
Krzysztof Pawlicki ◽  
Nikola Zmarzły ◽  
Magdalena Wójcik-Pędziwiatr ◽  
...  

Brain-derived neurotrophic factor (BDNF) is involved in the survival and maturation of neurons, and also promotes and controls neurogenesis. Its levels are lowered in many neurodegenerative diseases, including Huntington’s disease (HD). Clinical pictures of HD can be very diverse, which makes it difficult to assess its severity; however, molecular markers may be helpful. The aim of the study was to determine the relationship between HD severity and the plasma BDNF concentration in HD patients. The study recruited 42 patients with diagnosed and genetically confirmed HD and 40 healthy volunteers. BDNF levels were determined in plasma with the enzyme-linked immunosorbent assay (ELISA). Correlations between BDNF levels and clinical profiles and HD severity were also investigated. The BDNF level was significantly lower in HD patients compared to the control. There was no correlation between the BDNF level and motor symptoms and cognitive impairment. In the early disease stages, BDNF levels were associated with a better neurological examination, independence, and functional evaluation, in contrast to later HD stages, where the correlations were inverse. Multidirectional correlations between parameters of saccadic disorders and the BDNF level do not allow for drawing a conclusion, whether or not there is a relationship between the severity of saccadic disorders and the BDNF concentration.


2018 ◽  
Vol 8 (9) ◽  
pp. 161 ◽  
Author(s):  
Elisa Unti ◽  
Sonia Mazzucchi ◽  
Daniela Frosini ◽  
Cristina Pagni ◽  
Gloria Tognoni ◽  
...  

This study is aimed at relating social cognition in Huntington’s Disease (HD) to plasma levels of the social hormone oxytocin (OT). Indeed, HD patients commonly display reduced social skills and OT is involved in bonding behavior and improved recognition of facial emotions. Twelve mild-symptomatic HD patients (stage II Shoulson & Fahn) and 11 gender/age matched controls (healthy controls, HC), without concurrent psychiatric disorders, were investigated at baseline (T0) for OT plasma levels and social cognition through an extensive battery of neuropsychological tests. Social cognition was also re-examined after two years (T1) in 8 of the 12 patients. Results showed a trend for reduced T0-OT levels in HD vs. HC, mean ± stardard deviation: 6.5 ± 2.4 vs. 9.9 ± 7.2 pg/mL, without reaching statistical significance. At T0, patients showed significantly lower performances than controls at the “Faux-Pas” and “Strange Stories” tests (p < 0.05; p < 0.01); a reduced perception of visual emotions (p < 0.01) and verbal stimuli (p < 0.01) was also reported, involving anger, fear, and sadness (p < 0.05; p < 0.01). Additionally, in the HD population, OT concentrations positively correlated with T1-performances at Neutral\Faux-Pas test (p < 0.05), whereas the cognitive Montreal Cognitive Assessment (MoCA) and Mini Mental State Examination (MMSE) scores positively correlated with psychosocial perception at the “Strange Stories” and Karolinska Directed Emotional Faces (KDEF) tests (p < 0.05). This study, despite its limitations, supports correlations between OT and HD social cognition, suggesting a possible therapeutic use of this hormone. More subjects and additional body tissues/fluids, such as cerebrospinal fluid, should be investigated to confirm this hypothesis.


2004 ◽  
Vol 10 (2) ◽  
pp. 239-245 ◽  
Author(s):  
MEGHAN C. CAMPBELL ◽  
JULIE C. STOUT ◽  
PETER R. FINN

We examined the possible role of autonomic activity in Huntington's disease (HD) during a risky decision making task. Skin conductance responses (SCRs) of 15 HD participants and 16 healthy controls were measured while they performed a computerized version of the Simulated Gambling Task (SGT). The results replicated our previous finding of a performance decrement in HD, and showed that HD was associated with an altered pattern of SCRs during the risky decision task. Specifically, the healthy controls produced increased SCRs following selections from the disadvantageous decks and following losing selections. In contrast, the SCRs of the HD group did not differentiate between wins and losses. These findings indicate a reduced impact of loss on decision-making processes under risky conditions in HD. (JINS, 2004, 10, 239–245.)


2015 ◽  
Vol 22 (4) ◽  
pp. 426-435 ◽  
Author(s):  
Nelleke C. van Wouwe ◽  
Kristen E. Kanoff ◽  
Daniel O. Claassen ◽  
K. Richard Ridderinkhof ◽  
Peter Hedera ◽  
...  

AbstractObjectives: 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)


2007 ◽  
pp. 513-526 ◽  
Author(s):  
V Pérez-De La Cruz ◽  
A Santamaría

Huntington's disease (HD) is a demential, neurodegenerative inheritable disease affecting middle-aged patients. HD is characterized by uncontrolled choreiform movements, psychiatric symptoms and cognitive decline. Histopathological changes in HD brains reveal a considerable damage to basal ganglia, particularly affecting middle-sized spiny neurons from the caudate-putamen region. Neurochemical changes are specifically oriented to deplete GABAergic and cholinergic systems, while molecular alterations include an increased expression of CAG trinucleotide at exon 1 from the huntingtin (htt) gene, as well as aggregation of mutant htt. Although several hypotheses regarding the mechanisms by which neurotoxicity is triggered in HD brains have been suggested on the basis of experimental evidence, so far it remains not clear which of them are predominant or whether they are complementary. Recent experimental evidence through transgenic mice models reveal an interesting interaction between expanded CAG triplets, mutant htt, and the increase in toxic metabolites from the kynurenine pathway. Further evidence supports the assumption that different toxic mechanisms (i.e. excitotoxicity, energy metabolism impairment, inflammatory events, oxidative stress, etc.) are confluent and depend on each other. In this review we will briefly summarize some of those findings and propose a final integrative hypothesis for HD.


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