Reversible Cerebral Vasoconstriction Syndrome
Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by severe recurrent headaches (“thunderclap headaches”) with or without focal neurologic deficits and/or seizures as well as segmental constriction of cerebral arteries that resolves within 3 months. Visual complaints are frequent and include light sensitivity, blurred vision, and hemianopic visual field loss. This syndrome mainly affects middle-aged women, and more than one-half of RCVS cases occur during the postpartum period or after exposure to vasoactive substances (eg, selective serotonin reuptake inhibitors, triptans, amphetamines, ergotamine, nasal decongestants, or cannabis). Although the syndrome may be self-limited and benign, it may lead to ischemic stroke or nonaneurysmal subarachnoid hemorrhage. MRA or conventional angiography may demonstrate the characteristic “string-of-beads” appearance of the cerebral arteries. Calcium channel blockers are the standard therapies, but these have no proven effect on the syndrome’s hemorrhagic and ischemic complications (Fig 14-18).
Figure 14-18 Reversible cerebral vasoconstriction syndrome (RCVS) in a 34-year-old woman presenting with headache and a right homonymous hemianopia. A, Axial T2-weighted brain MRI shows a bilateral occipital infarct greater on the left than the right. B, Cerebral angiogram demonstrates multiple areas of segmental arterial narrowing (arrows).C, Areas of intracerebral vasoconstriction quickly improved 5 minutes after treatment with intravenous verapamil (arrows).
(Courtesy of Hormozd Bozorgchami, MD.)
Raven ML, Ringeisen AL, McAllister AR, Knoch DW. Reversible cerebral vasoconstriction syndrome presenting with visual field defects. J Neuroophthalmol. 2016;36(2):187–190.
Excerpted from BCSC 2020-2021 series: Section 5 - Neuro-Ophthalmology. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.