Brain Advance Access originally published online on April 16, 2004
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Brain, Vol. 127, No. 6, 1446-1453, 2004
© 2004 Guarantors of Brain
doi: 10.1093/brain/awh165
Neural correlates associated with impaired disgust processing in pre-symptomatic Huntingtons disease
1 Neurologische Klinik, 2 Nuklearmedizinische Klinik and 3 Institut für Röntgendiagnostik, Klinikum rechts der Isar, Technische Universität München, and 4 Institute of Experimental Psychology, Universität Regensburg, Germany
Correspondence to: Andreas Hennenlotter, Neurologische Klinik und Poliklinik, TU-München, Möhlstrasse 28, D-81675 München, Germany E-mail: a.hennenlotter{at}web.de
Disturbances in recognizing facial expressions of disgust have been reported previously in pre-symptomatic and manifest Huntingtons disease. Given the substantial role of the insula and basal ganglia in the perception of disgust as revealed by functional imaging, lesion studies and intracerebral recordings, we propose dysfunction within the insula and/or basal ganglia as the underlying neural substrate. Using functional MRI (fMRI), we studied a group of nine pre-symptomatic Huntingtons disease gene carriers and nine healthy controls, matched for age, gender, intelligence and years of education, while they were viewing disgusted facial expressions. As control conditions, surprised and neutral expressions were presented. Compared with healthy controls, Huntingtons disease gene carriers showed reduced responses within the left dorsal anterior insula during processing of disgusted facial expressions. Moreover, processing of disgust was associated with significant activation of the left dorsal anterior insula and putamen in healthy controls, but not in Huntingtons disease gene carriers. Furthermore, behavioural assessment revealed a selective impairment in recognizing facial expressions displaying disgust in Huntingtons disease gene carriers. Our finding of dysfunctional decreased insula activation in pre-symptomatic Huntingtons disease provides an explanation for the clinical deficit in recognizing facial expression of disgust. Furthermore, it underscores the role of the insula in the emotion of disgust.
Key Words: Huntingtons disease; fMRI; disgust; facial expression; insula
Abbreviations: CI= caudate index; FDR = false discovery rate; fMRI = functional MRI; IQ = intelligence quotient; ROI = region of interest
Received November 18, 2003. Revised February 6, 2004. Accepted February 14, 2004.
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