Brain, Vol 120, Issue 11 2029-2038, Copyright © 1997 by Oxford University Press
JM Gray, AW Young, WA Barker, A Curtis and D Gibson
Face processing and facial expression recognition were investigated in the
earliest stages of Huntington's disease, by studying 40 people who
presented for genetic testing. Twenty-three of these 'at risk' individuals
turned out not to carry the gene for Huntington's disease (the AR- group).
Seventeen were found to be gene carriers (the AR+ group); 15 from genetic
testing, and two who showed signs of early stages of Huntington's disease.
A number of standard tasks were used to provide background information,
including recognition memory for words, picture naming, verbal fluency, and
figure copying; none revealed significant differences between the AR+ and
AR- groups. Face processing abilities were investigated using tests of
identification of familiar (famous) faces, unfamiliar face matching,
recognition memory for faces, and recognition of facial expressions of
emotion. No statistically significant differences between the AR+ and AR-
groups were found for any of these tests, but the AR+ group showed a
borderline overall impairment in recognizing facial expressions of emotion
(0.05 < P < 0.1). When recognition of each of the six basic emotions
used was examined separately, only disgust was found to be significantly
impaired. This highly selective deficit in the recognition of disgust was
confirmed in the subgroup of 15 individuals shown by genetic testing to be
Huntington's gene carriers; it was therefore found in people who were free
from clinical symptoms and did not perform significantly more poorly than
non-carriers on any of the background tests, on any of the other face
processing tasks, and even for recognition of any other basic emotion. This
points strongly to the importance of the basal ganglia in the emotion of
disgust.
ARTICLES
Impaired recognition of disgust in Huntington's disease gene carriers
Neurobehavioural Service, St Nicholas Hospital, Gosforth, UK.
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