Abstract
BackgroundHuntington'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 FindingsWe 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/SignificanceThe 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.
Highlights
Huntington’s disease (HD) is an autosomal dominant, monogentic neurological disorder causing a degeneration of the neostriatum
The goal of the present study is to investigate whether patients with HD show alterations of the Ne as shown in Parkinson’s Disease (PD)
Both groups did not differ with respect to the frequency of errors [HD: 20.7269.50] [controls: 25.33610.25] (F(1,21) = 1.24; p = .278)
Summary
Huntington’s disease (HD) is an autosomal dominant, monogentic neurological disorder causing a degeneration of the neostriatum. The most obvious sign of HD is chorea: rapid, arrhythmic and complex involuntary movements, which are supposed to be an effect of dysfunctional error-feedback processing [3]. Besides these motor symptoms, psychiatric and cognitive deterioration appear, associated with dementia [4]. 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. Since dopaminergic alterations are related to the CAG-repeat, a Ne reduction may be related to the genetic disease load. The relation to the CAGrepeat expansion indicates that the Ne could serve as a gene-associated ‘‘cognitive’’ biomarker in HD
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
