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Brain Advance Access published online on April 16, 2004
Brain, doi:10.1093/brain/awh165
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Received November 18, 2003
Revised February 6, 2004
Accepted February 14, 2004

Article

Neural correlates associated with impaired disgust processing in pre-symptomatic Huntington’s disease

A. Hennenlotter 1*, U. Schroeder 2, P. Erhard 3, B. Haslinger 2, R. Stahl 2, A. Weindl 2, H. G. von Einsiedel 2, K. W. Lange 4, A. O. Ceballos-Baumann 2

1 Neurologische Klinik, Klinikum rechts der Isar, Technische Universität München, Germany; Neurologische Klinik und Poliklinik, TU-München, Möhlstrasse 28, D-81675 München, Germany
2 Neurologische Klinik, Klinikum rechts der Isar, Technische Universität München, Germany
3 Neurologische Klinik, Klinikum rechts der Isar, Technische Universität München, Germany; Nuklearmedizinische Klinik, Klinikum rechts der Isar, Technische Universität München, Germany; Institut für Röntgendiagnostik, Klinikum rechts der Isar, Technische Universität München, Germany
4 Institute of Experimental Psychology, Universität Regensburg, Germany

* To whom correspondence should be addressed. E-mail: a.hennenlotter{at}web.de.


  Abstract

Disturbances in recognizing facial expressions of disgust have been reported previously in pre-symptomatic and manifest Huntington’s 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 Huntington’s 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, Huntington’s 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 Huntington’s disease gene carriers. Furthermore, behavioural assessment revealed a selective impairment in recognizing facial expressions displaying disgust in Huntington’s disease gene carriers. Our finding of dysfunctional decreased insula activation in pre-symptomatic Huntington’s 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: Keywords: Huntington’s disease; fMRI; disgust; facial expression; insula


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