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From the literature on TS, it can be seen that children, adolescents and adult women with TS experience a range of social adjustment difficulties. These would appear NOT to be attributable to their short stature, odd physical appearance or infertility (McCauley, Kay, Ito, & Trader, 1987; Skuse et al., 1994). However, more needs to be understood about the nature of these difficulties. Reviewing the data from the pre-existing TS study database at the Institute of Child Health (see below) gave some valuable insights into the ways in which the social difficulties are manifest.

1.2.8.1 Autistic symptomatology

The Turner syndrome database at the Institute of Child Health, London is a nationwide record of children and adults with TS. It has been running since 1994 (Skuse et al., 1994) and now has data on almost 1,000 individuals with the syndrome. In the first piece of analysis reported in this thesis, adult

women with monosomie TS (45,X) were compared to age and Verbal IQ matched control women on the Autism Quotient Self-report Questionnaire (Baron-Cohen, Wheelwright, Skinner, Martin, & Clubley, 2001).

The Autism Quotient Self-report Questionnaire assesses the extent to which adults of normal intelligence possess traits that are characteristic of autism. It’s validity in discriminating individuals with autism from non-autistic

individuals has been established (Baron-Cohen et al., 2001). Sixty-one women with TS and twenty-six control females were assessed. Figure 1.1 shows that women with TS display significantly more autistic-like traits than a control group of women, having elevated total scores (t (1, 85) = -3.17,

XX (controls) XD (TS)

Figure 1.1; Mean total scores for control (XX) and Turner syndrome (XO) individuals on the Autism Quotient Self-report Questionnaire. Bars show mean. Error bars show standard error.

The clinical cut-off for this scale is 32+, with three (5%) of the women with TS obtaining scores above this criterion. None of the control females or the females in the original standardisation study obtained scores above the

clinical cut-off (Baron-Cohen et al., 2001). Women with TS generally displayed more autistic-like traits. Thirty three percent of the TS women endorsed twenty or more of the fifty items compared to twelve percent of the females without TS.

Analysis of the separate subscales revealed that these differences reached significance in two domains; namely social skill (F (1,85) = 13.05, p< 001) and imagination (F (1, 85) = 8.61, p<.004). The scale on which the TS group was

most severely affected was in the domain of social skills. TS women endorsed an average of nearly three out of ten items while control women endorsed less than one.

The individual items comprising the social skills subscale were analysed using Chi-square statistics, revealing that half of the items were more significantly endorsed (indicating an autistic-like trait) by the women with TS than by the control group (see table 1.1). Of these, the proportion of each group agreeing with the Items remained significantly different (after Bonferroni corrections for multiple comparisons) for two of the items. Forty two percent of the women with TS indicated that they found it hard to make new friends compared to just 8 percent of the control group. A further item may provide an insight into the precise nature of the social difficulties of the TS women. Nearly all (96%) of the control women indicated that they found it easy to work out what someone was thinking or feeling just by looking at their face compared to just 65% of the TS women.

Table 1.1: Autism self-report questionnaire - response rates for items comprising the social' subscale. Proportion item agreement (%) Item _Control females Turner syndrome Chi-Square

1.1 prefer to do things with others rather than on my own.

80.0 81.0 n/s

11.1 find social situations easy. 88.0 58.7 P=.008 13.1 would rather go to a library than a

party.

4.0 12.7 n/s

15.1 find myself more strongly drawn to people than to things.

96.0 81.0 n/s

22.1 find it hard to make new friends. ' 8.0 42.9 P=.002* 36.1 find it easy to work out what someone

is thinking or feeling just by looking at their 96.0 65.1 P=.003* face.

44.1 enjoy social occasions. 92.0 87.3 n/s

45.1 find it difficult to work out people’s intentions. *

20.0 47.6 P=.017

47.1 enjoy meeting new people. 96.0 84.1 n/s

48.1 am a good diplomat. 96.0 76.2 P=.03

^Significant at p<.05 after bonferroni corrections for multiple comparisons. ^ For these items, agreement is indicative of an autistic-like trait. On all other items in the table, disagreement is indicative of such traits.

Women with TS report that they do not find it easy to work out what someone Is thinking or feeling just by looking at their face. In clinical populations other than Turner syndrome face and emotion recognition deficits have been known to co-occur with difficulties in social functioning (Calder et al., 1996; Langdell,

1978; McConachie, 1976). Might there be something critical about the link between understanding and interpreting faces and facial expressions and typical social adjustment (Elgar & Campbell, 2001)? One of the hypotheses derived from this literature review will be that face and emotion recognition will be associated with poor social adjustment in women with TS.

Is there any evidence in the existing cognitive literature that women with Turner syndrome may have difficulties in processing faces, particularly concerning the emotional content of these facial displays?