Menopausal hormone therapy in women with CADASIL: a health system-wide retrospective cross-sectional study

Background: Menopausal hormone therapy (HT) alleviates menopause symptoms but may alter stroke risk and migraines. Concerns of compounding risk in patients with CADASIL may deter physicians from prescribing HT. We aimed to describe HT use patterns in women with CADASIL. Methods: We reviewed women >45 years with genetically or dermato-pathologically confirmed CADASIL. Clinical features, menopause symptoms, and HT use were collected from the electronic health record across Mayo Clinic. Characteristics were compared between non-HT users and HT users using the Wilcoxon rank-sum test for continuous variables and Fisher's exact test for categorical variables. Results: Among 89 CADASIL patients, 45 met demographic criteria. Of these, 10 (22.2 %) ever received HT and 35 (77.8 %) did not. There was no significant age difference between HT and non-HT users (53.3 +/- 11.8 vs. 54.1 +/- 8.3 years; P > 0.05). Migraine history was more common in HT users (100.0 % vs. 51.4 %; P = 0.007). Menopause symptoms were documented in 48.6 % of non-HT users, but HT use was discussed in only 23.5 %. Among HT users, non-systemic local vaginal formulations were most common (60.0 %), followed by the systemic transdermal (30.0 %). In follow-up, 50 % of patients either changed formulations or stopped HT. CADASIL was noted as a reason for the change in 40 %. Conclusions: Many CADASIL patients experiencing menopause symptoms did not receive HT. About one-fourth of women received HT, most commonly with non-oral formulations. Transdermal and vaginal formulations and other non-hormonal medications used to treat vasomotor symptoms may be safer than oral HT for women with CADASIL.