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The Journal of Neuroscience, November 5, 2008, 28(45):11695-11702; doi:10.1523/JNEUROSCI.2659-08.2008

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Behavioral/Systems/Cognitive
Increased Cognitive Functioning in Symptomatic Huntington's Disease As Revealed by Behavioral and Event-Related Potential Indices of Auditory Sensory Memory and Attention

Christian Beste,^1,2 Carsten Saft,³ Onur Güntürkün,⁴ and Michael Falkenstein¹

¹Institut für Arbeitsphysiologie an der Universität Dortmund–Leibniz Research Centre for Working Environment and Human Factors, D-44139 Dortmund, Germany, ²Department of Clinical Radiology, University of Münster, D-48149 Münster, Germany, ³Department of Neurology, Huntington Centre NRW, St. Josef-Hospital, Ruhr-University Bochum, D-44791 Bochum, Germany, and ⁴Biopsychology, Institute for Cognitive Neuroscience, Faculty of Psychology, Ruhr-University Bochum, 44780 Bochum, Germany

Correspondence should be addressed to Christian Beste, Institut für Arbeitsphysiologie an der Universität Dortmund-Leibniz Research Centre for Working Environment and Human Factors, Ardeystrasse 67, D-44139 Dortmund, Germany. Email: beste{at}ifado.de

Cognitive functions are thought to deteriorate globally in late stages of various neurodegenerative disorders. Here we describe that this general assumption is not justified and fails in Huntington's disease (HD). Presymptomatic gene mutation carriers (pHDs) and healthy controls performed worse compared with symptomatic HDs in an auditory signal detection task. During task performance, behavioral data and event-related potentials (ERPs) [i.e., MMN (mismatch negativity), P3a, and RON (reorienting negativity)] were recorded. Not only behavioral performance but also neurophysiological correlates of auditory sensory memory and attentional reorientation indicate enhanced performance occurring primal in late stages of a neurodegenerative disorder. Increased activity of the NMDA-receptor system, an assumed pathogenic mechanism in HD, might facilitate signal propagation at striatal level that enables more efficient task execution through a winner-take-all process. The results challenge the view that late stage neurodegeneration is necessarily related to a global decline in cognitive abilities in HD. In contrast, selectively enhanced cognitive functioning can emerge together with otherwise impaired cognitive functioning.

Key words: mismatch negativity (MMN); P3a; reorienting negativity (RON); sensory memory; attention; NMDA receptors; neurodegeneration; Huntington's disease (HD)

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Received June 11, 2008; revised Sept. 21, 2008; accepted Sept. 25, 2008.

Correspondence should be addressed to Christian Beste, Institut für Arbeitsphysiologie an der Universität Dortmund-Leibniz Research Centre for Working Environment and Human Factors, Ardeystrasse 67, D-44139 Dortmund, Germany. Email: beste{at}ifado.de

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