Neuropsychologia 41 (2003) 894–905 Interpreting gaze in Turner syndrome: impaired sensitivity to intention and emotion, but preservation of social cueing Kate Lawrence a , Ruth Campbell b,∗ , John Swettenham b , Janneke Terstegge c , Rebecca Akers a , Michael Coleman b , David Skuse a a Behavioural and Brain Sciences, Institute of Child Health, University College London, London, UK b Department of Human Communication Science, University College London, Chandler House, 2 Wakefied Street, London WC1N 1PF, UK c Department of Psychonomics, University of Utrecht, Utrecht, The Netherlands Received 27 November 2002; received in revised form and accepted 11 December 2002 Abstract Women with Turner’s syndrome (TS), who lack a complete X-chromosome, show an impairment in remembering faces and in classifying ‘fear’ in face images. Could their difficulties extend to the processing of gaze? Three tasks, all of which rely on the ability to make use of the eye-region of a pictured face, are reported. Women with TS were impaired at judging mental state from images of the upper face (‘reading the mind in the eyes’). They were also specifically impaired at interpreting ‘fear’ from displays of the eye-region of the face. However, they showed normal susceptibility to direction of gaze as an attentional cue (social cueing), since they were as sensitive as controls to the validity of the cue, under conditions where it should be ignored. In this task, unlike those of reading the upper face for intention or expression, PIQ accounted for a significant amount of individual variance in task performance. The processing of displays of the eye region affording social and affective information is specifically affected in TS. We speculate that amygdala dysfunction is likely to be implicated in this anomalous behaviour. The presence in the female karyotype of two complete X-chromosomes is protective for some socio-cognitive abilities related to the modulation of behaviour by the interpretation of gaze. © 2003 Elsevier Science Ltd. All rights reserved. 1. Introduction Turner’s syndrome (TS) [73] is a sporadic disorder of human females in which all or part of one X-chromosome is deleted. Such individuals are phenotypically female, but they have short stature and, in the absence of normal ovarian development, fail to develop secondary sexual characteris- tics. Growth hormone treatment is often administered dur- ing childhood, and oestrogen replacement therapy is given routinely in adolescence. Verbal intelligence in TS is typically normal [50], and although visuospatial skills may often be poor [64,65,72], there is no increased risk of general mental retardation. While their social development does not cause comment in a recent clinical review of the syndrome [66], there are, nevertheless, reports that adult women with TS may have problems forming and maintaining peer relationships, and difficulties interpreting non-verbal communication [28,51,52]. Some researchers report that the condition can ∗ Corresponding author. Tel.: +44-20-7679-4200; fax: +44-20-7713-0861. E-mail address: r.campbell@ucl.ac.uk (R. Campbell). be associated with psychosocial difficulties, immaturity, and poor self-concept [52,53,68]. We have recently reported that such difficulties can also be demonstrated in laboratory tasks. Women with TS can be impaired at recognising faces and identifying facial expressions of emotion, especially the classification of ‘fear’ in faces [48,62,64,65]. Moreover, in our clinical interviews with parents of children and adolescents with TS we observed that girls and women with TS rarely engaged gaze with the in- terviewer, and sometimes did not glance at a caregiver entering the room. This pattern of behaviours: lowered levels of face recognition, impaired processing of ‘fear’ in faces, and anecdotal observations of deficient eye-contact in social encounters, are reminiscent of similar behaviours reported in people with autism [14,24,26,41,45]. A further well-established finding in autism research is defective ability in perceptual processing of the eye-region of faces [9,36,42,46]. The aim of the present study was to explore the ex- tent to which women with TS may show anomalies in the processing of eye-regions of the face in order to derive information about peoples intentions and emotions. Three tasks are reported. The first required the interpretation and 0028-3932/03/$ – see front matter © 2003 Elsevier Science Ltd. All rights reserved. doi:10.1016/S0028-3932(03)00002-2