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. 2006 Dec 20;1(1):e86.
doi: 10.1371/journal.pone.0000086.

Error processing in Huntington's disease

Christian Beste  1 , Carsten Saft, Jürgen Andrich, Ralf Gold, Michael Falkenstein
Affiliations

Error processing in Huntington's disease

Christian Beste et al. PLoS One. .

Abstract

Background: Huntington's disease (HD) is a genetic disorder expressed by a degeneration of the basal ganglia inter alia accompanied with dopaminergic alterations. These dopaminergic alterations are related to genetic factors i.e., CAG-repeat expansion. The error (related) negativity (Ne/ERN), a cognitive event-related potential related to performance monitoring, is generated in the anterior cingulate cortex (ACC) and supposed to depend on the dopaminergic system. The Ne is reduced in Parkinson's Disease (PD). Due to a dopaminergic deficit in HD, a reduction of the Ne is also likely. Furthermore it is assumed that movement dysfunction emerges as a consequence of dysfunctional error-feedback processing. Since dopaminergic alterations are related to the CAG-repeat, a Ne reduction may furthermore also be related to the genetic disease load.

Methodology/principle findings: We assessed the error negativity (Ne) in a speeded reaction task under consideration of the underlying genetic abnormalities. HD patients showed a specific reduction in the Ne, which suggests impaired error processing in these patients. Furthermore, the Ne was closely related to CAG-repeat expansion.

Conclusions/significance: The reduction of the Ne is likely to be an effect of the dopaminergic pathology. The result resembles findings in Parkinson's Disease. As such the Ne might be a measure for the integrity of striatal dopaminergic output function. The relation to the CAG-repeat expansion indicates that the Ne could serve as a gene-associated "cognitive" biomarker in HD.

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Conflict of interest statement

Competing Interests: The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Event-related potentials of the Ne/ERN and CRN.
The Ne/ERN (false responses) and CRN (correct responses) for HD and controls at electrode FCz (top) and at Fz (bottom). The x-axis denotes time in milliseconds (ms). The y-axis denotes voltage in µV. The bar plots denote significant differences in the peak-to-peak amplitude of the Ne/ERN between the groups for the electrodes FCz (left) and Fz (right).
Figure 2
Figure 2. The Relation of the Ne peak-to-peak amplitude at Fz and CAG-index.
This figure shows a linear relation of the Ne peak-to-peak amplitude at electrode Fz with the CAG-index, derived via (CAGn – 35.5) × “age of the patient” [see: 2], .
See this image and copyright information in PMC

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