112. Reversible cerebral vasoconstriction syndrome associated with pregnancy in peripartum period

Abstract

Introduction Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by reversible multifocal narrowing of the cerebral arteries, generally preceded by thunderclap headaches with or without associated neurological deficits. RCVS develops a unique set of clinical imaging. Though RCVS is typically considered benign and self-limited disease, some reports showed that the patients with RCVS had a higher proportion of non-benign outcomes. Here, we present 6 cases of RCVS associated with pregnancy. Methods Patients were retrospectively identified at TOYOTA Memorial Hospital, from 2010 to 2017. We diagnosed RCVS based on the presence of reversible vasoconstrictions of cerebral arteries in magnetic resonance angiography (MRA) and neurological symptoms in peripartum period. These vasoconstrictions disappeared in the follow-up MRA within 3 months. Clinical course of these patients were collected. Results The average age was 36.8 years old and 4 patients were primipara. Four patients were complicated with preeclampsia or eclampsia. Headache was the initial symptom in 3 patients and only 1 patient developed typical thunderclap headache. Four patients developed RCVS in peripartum period, and 2 patients in postpartum period. Intracranial hemorrhage and non-aneurysmal subarachnoid hemorrhage were detected in 1 patient respectively and PRES was detected in 3 patients. Magnesium sulfate was administered in 2 patients who presented with convulsion related to intracranial hemorrhage or eclampsia. No patient developed neurological sequela after the resolution of vasoconstrictions. Discussion Frequency of a typical thunderclap headache is relatively low in RCVS associated with pregnancy. RCVS can be associated with PRES and eclampsia closely. Prompt recognition and early imaging studies are essential for the diagnosis of RCVS associated with pregnancy.