Plasma testosterone, dehydroepiandrosterone sulfate, and cortisol in female patients with Huntington's disease.

OBJECTIVE: The neuronal loss in several brain regions that characterize the progression of Huntington's disease (HD), is expected to influence the activities of hypothalamus-adrenal and hypothalamus-gonadal axes, and the changes may relate to common features of the disease, like depression and dementia. While in male HD patients low plasma testosterone levels have been reported, information on female patients is lacking.

METHODS: We assessed the plasma levels of the androgens total testosterone (TT) and dehydroepiandrosterone sulfate (DHEAS), as well as of cortisol in 41 female patients with HD, confirmed by determination of the CAG repeat number in the IT-15 gene, and searched for associations to the disease symptomatology. We also included a group of 18 females with expanded CAG repeat number in the HD gene (subjects at risk), and a group of 66 age-matched healthy females. Hormone levels of the pre- and post-menopausal subgroups were also compared separately.

RESULTS: Significant negative correlations to age were found for TT and DHEAS in both control (age range 20-71 years) and patient (age range 26 to 78 years) groups, and the calculated decline per year was around 1% for TT and 1.5% for DHEAS. There were no significant differences in hormone levels among patients, subjects at risk and controls, either in premenopausal or in postmenopausal state. The subgroup of patients with depression in their symptomatology had significantly lower TT and DHEAS levels compared to patients without depression, or to controls.

CONCLUSIONS: While TT and DHEAS seem to decline with age in female patients with HD to the same extend as for healthy females, the presence of depression, but not dementia, in their symptomatology, is connected to lower ovary-adrenal androgen levels.