Stimulus-Response Compatibility in Huntington's Disease: A Cognitive-Neurophysiological Analysis

Abstract

Beste C, Saft C, Andrich J, Gold R, Falkenstein M. Stimulusresponse compatibility in Huntington's disease: a cognitive-neurophysiological analysis. J Neurophysiol 99: 1213-1223, 2008. First published January 9, 2008 doi:10.1152/jn.01152.2007. The basal ganglia are assumed to be of importance in action/response selection, but results regarding the importance are contradictive. We investigate these processes in relation to attentional processing using eventrelated potentials (ERPs) in Huntington's disease (HD), an autosomal genetic disorder expressed by degeneration of the basal ganglia, using a flanker task. A symptomatic HD group, a presymptomatic HD group (pHD), and healthy controls were examined. In the behavioral data, we found a general response slowing in HD while the compatibility effect was the same for all groups. The ERP data show a decrease of the N1 on the flanker in HD and pHD; this suggests deficient attentional processes. The N1 on the target was unaffected, suggesting that the attentional system in HD is not entirely deficient. The early lateralized readiness potential (LRP), reflecting automatic response activation due to the flankers, was unchanged, whereas the late LRP, reflecting controlled response selection due to the target information, was delayed in HD. Thus levels of action-selection processes are differentially affected in HD with automatic processes seeming to be more robust against neurodegeneration. The N2, usually associated with conflict processing, was reduced in the HD but not in the pHD and the control groups. Because the N2 was related to the LRP and reaction times in all groups, the N2 may generally not be related to conflict but rather to controlled response selection, which is impaired in HD. Overall, the results suggest alterations in attentional control, conflict processing, and controlled response selection in HD but not in automatic response selection.

Emotion perception and electrophysiological correlates in Huntington’s disease

“…To our knowledge there are currently no published ERP emotion perception studies in HD. The use of ERPs has however been implemented in other areas of HD research (for review see Nguyen et al, 2010), where attenuated amplitudes (reduced coherent neural firing rates) across a range of ERP indices have been reported (e.g., Antal et al, 2003, Beste et al, 2008, Munte et al, 1997. Emotional faces can similarly be studied using ERPs.…”

Computational Investigations of Cognitive Impairment in Huntington's Disease

“…The response time in the incongruent condition is slower than in the congruent condition, which is explained by the interference caused by the distractors. Huntington's patients are generally slower in this task, but do not show an abnormal interference effect (Beste, et al, 2008).…”

“…Interestingly, the CAG-index of HD patients correlated with the size of the Ne/ERN response (Beste, et al, 2006). Compared to HD, pHD showed stronger brain responses, but not more than controls, apart from errorrelated processing (Beste, et al, 2007(Beste, et al, , 2008. It has been suggested that pHD may exhibit compensatory activation in other brain regions (Feigin, et al, 2006;Paulsen, et al, 2004).…”

“…Apart from behavioral measures, Beste et al (2006Beste et al ( , 2007Beste et al ( , 2008 recorded electrophysiological responses and observed that compared to controls, HD showed reduced stimulus-related potentials (i.e., N1), slowed motor-related components (i.e., lateralized readiness potentials), and reduced error-related processing (i.e., Ne/ERN). Interestingly, the CAG-index of HD patients correlated with the size of the Ne/ERN response (Beste, et al, 2006).…”

Basal Ganglia and the Error Monitoring and Processing System: How Alcohol Modulates the Error Monitoring and Processing Capacity of the Basal Ganglia

“…For instance, patients with Huntington's disease show great decrease in the ability to assess various aspects of tasks (disorder in attention), resulting from a disorder in action-selection processes. This obviously will lead to increase in error commission in tasks (Beste et al, 2008). One recent study by Bocquillon and co-workers (2012) suggests that basal ganglia pathologies results in resistance impairment to distracters (which might actually, in most cases represent competing neural information), hence providing more grounds for error commission.…”