Gender and hormonal influences in reversible cerebral vasoconstriction syndrome

The reversible cerebral vasoconstriction syndromes, including postpartum angiopathy, have been characterized over the last decade. Women are predominantly affected. Some studies suggest that postpartum angiopathy carries a worse prognosis.

We compared the clinical, neuroimaging, and angiographic features of 36 men, 110 non-pregnant women and 16 postpartum women included in our single-center cohort of patients with reversible cerebral vasoconstriction syndromes encountered from 1998 to 2016.

As compared to men, non-pregnant women were older (48 ± 11 vs. 34 ± 13 years, p < 0.001), had more underlying migraine (49% vs. 19%, p = 0.002), depression (53% vs. 14%, p < 0.001) and serotonergic antidepressant use (45% vs. 11%, p < 0.001), developed more clinical worsening (18% vs. 3%, p = 0.022), more infarcts (39% vs. 20%, p = 0.031) and worse angiographic severity scores (23 ± 14 vs. 10.9 ± 10.3, p < 0.001), but had similar discharge outcomes (modified Rankin scale scores 0-3, 90% vs. 91%, p = 0.768). Sexual activity was an important trigger in men (22% vs. 4%, p = 0.002). As compared to non-pregnant women, postpartum angiopathy patients were younger (33 ± 6 years, p < 0.001) and had less vasoconstrictive drug exposure (25% vs. 67%, p = 0.002) but showed similar clinical, radiological and angiographic findings and similar discharge outcomes (modified Rankin scale scores 0-3 in 94%, p = 0.633). There were no significant differences between pre- and post-menopausal women, or those with and without hysterectomy.

The observed gender differences in reversible cerebral vasoconstriction syndromes may result from hormonal or non-hormonal factors. Hormonal imbalances may trigger reversible cerebral vasoconstriction syndromes. Given the absence of significant differences in the female subgroups, hormonal factors do not appear to significantly affect the course or outcome of reversible cerebral vasoconstriction syndromes.