Reversible cerebral vasoconstriction syndrome in patients with polycythemia vera

Abstract

Introduction: Reversible cerebral vasoconstriction syndrome (RCVS) may occur spontaneously, especially in middle-aged women but half the cases are associated with nonaneurysmal subarachnoid hemorrhage, post partum, or vasoactive drugs. RCVS has been also known to occur with diverse clinical diseases but this is the first report to implicate polycythemia vera (PV).

Case report: A 54-year-old woman presented with recurrent thunderclap headaches, aggravated by coughing. She was diagnosed PV 3 years ago and well controlled with aspirin and hydroxyurea. A bone marrow biopsy and a positive JAK-2-V617F mutation provided a diagnosis of PV. She did not take any other medication except aspirin and hydroxyurea in the last 2 months. Her neurologic examination was normal. Brain CT angiography 6 days after symptoms onset showed multiple cerebral arterial irregularities with segmental narrowing and dilatation. Cerebral angiography confirmed multifocal segmental narrowing and dilatation in the same vascular territories. She was treated with oral nimodipineand her symptoms were improved within 3 weeks. Follow-up brain MR angiography 3 months after symptoms onset demonstrated normalization of involved vessels. She had no headache during 12 months follow-up.

Conclusion: Thrombosis does not account for RCVS in patients with PV but endothelial damage from repetitive microvascular thrombosis may be associated with RCVS. In this patient, prothrombotic state affecting to endothelial damage might cause a transient disturbance in the regulation of cerebral vascular tone.