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

Abstract

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 [Figure
1(B)].
In order to better define the extent of arterial pathology
and to exclude other potential causes of the patient’s
clinical and radiological findings, a digital subtraction
cerebral angiogram (DSA) was then performed. The
DSA demonstrated extensive and severe beading of
large and medium caliber intracranial arteries; multiple
segments of smooth and high-grade stenosis were interspersed with normal caliber arterial segments [Figure
2(A) and (B)]. No intracranial aneurysm was identified
and the arteries of the external carotid artery circulation
were found to be normal.
Given that the DSA findings suggested RCVS and given
the severity of the arterial stenoses, 10mg of verapamil
was instilled into each internal carotid artery and into the
left vertebral artery. This administration of intra-arterial
calcium channel blockers produced a dramatic angiographic result with near complete resolution of the multifocal arterial stenoses [Figure 2(C)].
Although the patient’s headaches persisted for three
more days, her blood pressure normalized, she was
found to be neurologically intact and she was discharged
from hospital four days after the DSA.