Severe reversible cerebral vasoconstriction syndrome in a postpartum patient treated successfully with direct instillation of intra-arterial verapamil during cerebral angiography

A 36-year-old woman developed a sudden onset thunderclap headache and visual disturbance, while undergoing a caesarean section for her second pregnancy. Ten minutes after delivery, she suffered a sustained, generalized tonic-clonic seizure and required intubation and, following intubation, was transferred to the intensive care unit.

Following cessation of seizure activity, the patient was extubated and on examination was found to be neurologically intact. A noncontrast CT brain was unremarkable; however, intracranial MRI and MR angiography (MRA) demonstrated multifocal, low-grade arterial stenoses in several vascular territories without evidence of infarction or hemorrhage. The two major differentials of these MR findings were cerebral vasculitis and reversible cerebral vasoconstriction syndrome (RCVS), and given the patient’s postpartum presentation, RCVS was considered the most likely diagnosis. Four days after delivery, the patient was neurologically intact and experiencing only minor headaches and, therefore, was discharged on oral calcium cannel blockers for treatment of cerebral vasoconstriction and oral pregabalin for seizure prophylaxis.

One week later, the patient represented to hospital with severe headaches and was found to be markedly hypertensive with a systolic blood pressure over 190 mm Hg. Neurological examination identified bilateral agraphesthesia without other focal neurological deficits. Repeated MRI/MRA performed at this time demonstrated significant progression of disease; multiple bilateral cortical watershed infarcts [Figure 1(A)] had developed since initial MRI and multiple high-grade stenosis were now seen within the anterior, middle, and posterior cerebral arteries bilaterally and within the basilar artery