PT  - JOURNAL ARTICLE
AU  - Christian Beste
AU  - Carsten Saft
AU  - Onur Güntürkün
AU  - Michael Falkenstein
TI  - Increased Cognitive Functioning in Symptomatic Huntington's Disease As Revealed by Behavioral and Event-Related Potential Indices of Auditory Sensory Memory and Attention
AID  - 10.1523/JNEUROSCI.2659-08.2008
DP  - 2008 Nov 05
TA  - The Journal of Neuroscience
PG  - 11695--11702
VI  - 28
IP  - 45
4099  - http://www.jneurosci.org/content/28/45/11695.short
4100  - http://www.jneurosci.org/content/28/45/11695.full
SO  - J. Neurosci.2008 Nov 05; 28
AB  - 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.