A Population-Based Study of Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction Syndrome During Pregnancy and Puerperium.

BACKGROUND

Posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS) are related neurovascular conditions, with pregnancy as a shared risk factor. In this study, we aimed to describe shared and distinctive risk factors and outcomes in patients with pregnancy-associated PRES and/or RCVS.

METHODS

In this retrospective, nationwide, population-based cohort study, the national healthcare registers were utilized to identify women with PRES and/or RCVS during pregnancy or puerperium during 1987-2016. Subsequent pregnancies, vascular events, and deaths until 2022 were identified. Medical records were reviewed to classify cerebrovascular events and collect clinical details.

RESULTS

In total, 27 patients had pregnancy-associated PRES and/or RCVS (18 PRES; 5 PRES + RCVS; 4 RCVS) during 1987-2016, resulting in an incidence of 1.52 per 100,000 (95% Cl 1.02-2.18) deliveries. All patients with PRES ± RCVS had preeclampsia with severe features during late pregnancy or early puerperium. In contrast, isolated RCVS showed a weaker association to preeclampsia and occurred in late puerperium, with a median puerperal day of 26 (IQR 11-45). Altogether, 40.7% of all patients had a stroke, 90.9% of which were hemorrhagic. Preeclampsia was diagnosed in 90.9% of stroke patients. Maternal mortality was 3.7%, whereas perinatal mortality was 7.4%. At 3 months, 92.3% had a good recovery (mRS 0-2). During follow-up, stroke recurrence was 3.7% and 33.3% had subsequent uneventful, full-term pregnancies.

CONCLUSIONS

Pregnancy-associated PRES and RCVS are potentially life-threatening, rare conditions that can result in hemorrhagic stroke. PRES ± RCVS is strongly associated with preeclampsia with severe features, whereas puerperal RCVS seems to be a separate, later-occurring condition.