JOURNAL ARTICLE
MULTICENTER STUDY

Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease

Shannon A Johnson, Julie C Stout, Andrea C Solomon, Douglas R Langbehn, Elizabeth H Aylward, Christina B Cruce, Christopher A Ross, Martha Nance, Elise Kayson, Elaine Julian-Baros, Michael R Hayden, Karl Kieburtz, Mark Guttman, David Oakes, Ira Shoulson, Leigh Beglinger, Kevin Duff, Elizabeth Penziner, Jane S Paulsen
Brain: a Journal of Neurology 2007, 130 (Pt 7): 1732-44
17584778
Previous studies of emotion recognition suggest that detection of disgust relies on processing within the basal ganglia and insula. Research involving individuals with symptomatic and pre-diagnostic Huntington's disease (HD), a disease with known basal ganglia atrophy, has generally indicated a relative impairment in recognizing disgust. However, some data have suggested that recognition of other emotions (particularly fear and anger) may also be affected in HD, and a recent study found fear recognition deficits in the absence of other emotion-recognition impairments, including disgust. To further examine emotion recognition in HD, we administered a computerized facial emotion recognition task to 475 individuals with the HD CAG expansion and 57 individuals without. Logistic regression was used to examine associations of emotion recognition performance with estimated proximity to clinical diagnosis (based on CAG repeat length and current age) and striatal volumes. Recognition of anger, disgust, fear, sadness and surprise (but not happiness) was associated with estimated years to clinical diagnosis; performance was unrelated to striatal volumes. Compared to a CAG-normal control group, the CAG-expanded group demonstrated significantly less accurate recognition of all negative emotions (anger, disgust, fear, sadness). Additionally, participants with more pronounced motor signs of HD were significantly less accurate at recognizing negative emotions than were individuals with fewer motor signs. Findings indicate that recognition of all negative emotions declines early in the disease process, and poorer performance is associated with closer proximity to clinical diagnosis. In contrast to previous results, we found no evidence of relative impairments in recognizing disgust or fear, and no evidence to support a link between the striatum and disgust recognition.

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