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A Population-Based Study of Posterior Reversible Encephalopathy Syndrome and Reversible Cerebral Vasoconstriction
Vest Teresa1, Verho Liisa2, Rantanen Kirsi1
1Department of Neurology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Insights
Pregnancy-associated Posterior Reversible Encephalopathy Syndrome (PRES) and Reversible Cerebral Vasoconstriction Syndrome (RCVS) are rare, serious conditions. PRES with or without RCVS strongly links to severe preeclampsia, while isolated RCVS occurs later in the postpartum period.
Area of Science:
- Neuroscience
- Obstetrics
- Vascular Medicine
Background:
- Posterior Reversible Encephalopathy Syndrome (PRES) and Reversible Cerebral Vasoconstriction Syndrome (RCVS) are related neurovascular conditions.
- Pregnancy is a significant shared risk factor for both PRES and RCVS.
- Understanding shared and distinct risk factors and outcomes is crucial for managing these conditions during pregnancy and postpartum.
Purpose of the Study:
- To describe shared and distinct risk factors in patients with pregnancy-associated PRES and/or RCVS.
- To outline the clinical outcomes for patients diagnosed with pregnancy-associated PRES and/or RCVS.
- To differentiate the characteristics of PRES with RCVS versus isolated RCVS in the context of pregnancy.
Main Methods:
- Retrospective, nationwide, population-based cohort study.
- Utilized national healthcare registers to identify women with PRES and/or RCVS from 1987-2016.
- Reviewed medical records to classify cerebrovascular events and collect clinical details, including subsequent pregnancies and outcomes.
Main Results:
- Incidence of pregnancy-associated PRES and/or RCVS was 1.52 per 100,000 deliveries.
- All PRES±RCVS cases were associated with preeclampsia with severe features; isolated RCVS occurred later postpartum (median day 26).
- 40.7% experienced stroke (90.9% hemorrhagic), 3.7% maternal mortality, 7.4% perinatal mortality, with 92.3% good recovery at 3 months.
Conclusions:
- Pregnancy-associated PRES and RCVS are rare, potentially life-threatening conditions.
- PRES±RCVS is strongly linked to severe preeclampsia.
- Puerperal RCVS appears to be a distinct entity occurring later in the postpartum period.
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.
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