A Case of Postpartum Reversible Cerebral Vasoconstriction Syndrome with Extracranial Artery Involvement.

Abstract

Abstract: Reversible cerebral vasoconstriction syndrome (RCVS) is generally recognized as an intracranial pathology. Involvement of the extracranial segments of the carotid or vertebral arteries (VAs) is rare. Here, we present a case of postpartum RCVS with extracranial VA involvement. A 31-year-old female developed postpartum thunderclap headaches with neck pain, followed by generalized seizures. The patient had been healthy with an uncomplicated pregnancy and delivery. Head computed tomography showed minimal right frontal convexity subarachnoid hemorrhage. Transcranial color-coded sonography disclosed increased mean flow velocity in multiple arteries, consistent with vasoconstriction, while the extracranial Doppler study revealed an abrupt change in the diameter of the left VA, indicative of dissection or vasospasm. Brain magnetic resonance imaging demonstrated nonenhancing T2/fluid-attenuated inversion recovery hyperintensities involving bilateral occipital, parietal, and frontal regions, with normal diffusion-weighted imaging signals, suggesting vasogenic edema. Magnetic resonance angiography showed multifocal segmental vasoconstriction of cerebral arteries, along with multiple irregular luminal narrowing of bilateral extracranial VAs. T1 fat-suppressed images revealed no features indicative of arterial dissection. The patient was treated with nimodipine as she was diagnosed with postpartum RCVS with extracranial artery involvement and concomitant posterior reversible encephalopathy syndrome. During an outpatient follow-up 1 month after discharge, she reported complete resolution of symptoms. It is crucial to acknowledge that cervical artery vasoconstriction, while rare, remains a possibility in RCVS. In patients having significant multiple extracranial artery vasoconstriction, even when they are the main or even the only arteries involved, RCVS should always be taken into consideration.