Sexual development and life of women with gonadal dysgenesis.

The sexual development and behavior of 37 women suffering from the gonadal dysgenesis syndrome were investigated. It was found that their sexual development was significantly retarded compared with a control group of 50 fertile patients of the same age. This retardation was greater in a subgroup of 19 women with the Turner syndrome than in a subgroup of 18 women with pure gonadal dysgenesis. The levels of gonadotrophins LH and FSH, estradiol, progesterone and testosterone in blood were similar in these two subgroups. In two-thirds of the gonadal dysgenesis syndrome patients, the estradiol level was mostly at the low end of normal. The assessment of their sexual life using the Sexual Activity of Women (SAW) and Sexual Function of Women (SFW) questionnaires indicated a lower sexual desire, a reduced orgastic capacity, and a weaker sexual activity in the pathological group. However, 24 patients living in a permanent and stable partner relationship at the time of the examination, did not significantly differ from the control group in their responses to the SFW questionnaire. The patients with gonadal dysgenesis syndrome were surprisingly well adapted to their shortcomings. The occurrence and intensity of most neurotic symptoms assessed by the N5 Questionnaire were scored lower by the patients with gonadal dysgenesis syndrome than by the control group.