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J Neurophysiol 99: 1213-1223, 2008. First published January 9, 2008; doi:10.1152/jn.01152.2007
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Stimulus-Response Compatibility in Huntington's Disease: A Cognitive-Neurophysiological Analysis

Christian Beste1,2,3, Carsten Saft2, Jürgen Andrich2, Ralf Gold2 and Michael Falkenstein1

1Leibniz Research Centre for Working Environment and Human Factors, World Health Organization Collaborating Centre for Occupational Health and Human Factors, Dortmund; 2Department of Neurology, Huntington Centre NRW, St. Josef-Hospital, Ruhr-University Bochum, Bochum; and 3Department of Clinical Radiology, University of Münster, Munster, Germany

Submitted 18 October 2007; accepted in final form 3 January 2008

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 event-related 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.


Address for reprint requests and other correspondence: C. Beste, Leibniz Research Centre for Working Environment and Human Factors, WHO Collaborating Centre for Occupational Health and Human Factors, Ardeystr.67, D-44139 Dortmund, Germany (E-mail: beste{at}ifado.de)




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C. Beste, C. Saft, O. Gunturkun, and M. Falkenstein
Increased Cognitive Functioning in Symptomatic Huntington's Disease As Revealed by Behavioral and Event-Related Potential Indices of Auditory Sensory Memory and Attention
J. Neurosci., November 5, 2008; 28(45): 11695 - 11702.
[Abstract] [Full Text] [PDF]




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