Reversible cerebral vasoconstriction syndrome: an under-recognized clinical emergency

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches and reversible cerebral vasoconstrictions. RCVS is more common than previously thought and should be differentiated from aneurismal subarachnoid hemorrhage. RCVS can be spontaneous or evoked by pregnancy or exposure to vasoactive substances. Patients tend to be middle-aged women but pediatric patients have been seen. Up to 80% of sufferers have identifiable triggers. Thunderclap headaches tend to recur daily and last for a period of around 2 weeks, while the vasoconstrictions may last for months. About one-third of patients have blood pressure surges accompanying headache attacks. The potential complications of RCVS include posterior reversible encephalopathy syndrome, ischemic strokes over watershed zones, cortical subarachnoid hemorrhage and intracerebral hemorrhage. Magnetic resonance images including angiography and venography and lumbar punctures are the studies of choice, whereas catheter angiography should not be implemented routinely. Patients with a mean flow velocity of the middle cerebral artery greater than 120 cm/s shown by transcranial color-coded sonography have a greater risk of ischemic complications than those without. The pathophysiology of RCVS remains unknown; sympathetic hyperactivity may play a role. Open-label trials showed calcium channel blockers, such as nimodipine may be an effective treatment in prevention of thunderclap headache attacks. In severe cases, intra-arterial therapy may be considered. Most patients with RCVS recover without sequelae; however, relapse has been reported in a small proportion of patients.

Keywords: posterior reversible encephalopathy syndrome; reversible cerebral vasoconstriction syndrome; thunderclap headache.

Figure 1.

Cerebral vasoconstrictions (a) and their resolution (b) on magnetic resonance angiography in patients with reversible cerebral vasoconstriction syndrome.

Figure 2.

Posterior reversible encephalopathy syndrome on fluid-attenuated inversion recovery image.

Figure 3.

Proposed pathophysiology of reversible cerebral vasoconstriction syndrome. PRES, posterior reversible encephalopathy syndrome; TCH, thunderclap headache.