Reversible Cerebral Vasoconstriction Syndrome: a Comprehensive Update

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinico-radiological syndrome characterized by recurrent thunderclap headache, with or without neurologic symptoms, and reversible vasoconstriction of cerebral arteries. RCVS affects patients in various racial and ethnic groups and in all age groups, although most commonly in the fourth decade of life. Many conditions and exposures have been linked to RCVS, including vasoactive drugs and the peripartum period. Disturbance of the cerebral vascular tone is thought to contribute to the disease's pathophysiology. RCVS generally follows a monophasic course. Associated strokes and cerebral hemorrhages are not uncommon. In this review we will attempt to provide a comprehensive overview of RCVS, with emphasis on the controversies in the field and the newest findings in the reported literature.

Right Hemispheric Reversible Cerebral Vasoconstriction Syndrome in a Patient with Left Hemispheric Partial Seizures

“…Th ere is a clear female predilection, with a mean age of onset around 42 years (4). Th e headaches are recurrent in more than 95% of patients and occur over the course of 1 to 3 weeks (4,5). Th e headaches are described as bilateral, with posterior onset and subsequent generalized pain.…”

“…Th e headaches are described as bilateral, with posterior onset and subsequent generalized pain. Th ey can last for minutes to days with an average of four attacks (4). A single attack is uncommon (3).…”

“…Moderate headaches are seen between the more severe bouts, accompanied by nausea, vomiting, photophobia, and phonophobia (6). In 8% to 40% of patients, RCVS can also present with transient or persistent neurologic defi cits, including encephalopathy, dysarthria, ataxia, focal numbness, weakness, seizures, and visual changes such as scotomas, blurry vision, hemianopsia, and cortical blindness (3,4). Visual symptoms are the most commonly encountered.…”

“…CSF is signifi cant for a marked infl ammatory profi le and can also reveal oligoclonal bands. Brain magnetic resonance imaging may reveal deep infarcts and contrast enhancements of the vessel wall (3,4). It is of some importance to accurately distinguish these two entities, since treatment for PACNS with corticosteroids can be harmful in RCVS.…”

Reversible cerebral vasoconstriction syndrome and posterior reversible encephalopathy syndrome in a boy with Loeys‐Dietz syndrome

“…Reversible cerebral vasoconstriction syndrome (RCVS) is a clinico-radiological condition, characterized by recurrent sudden-onset severe "thunderclap" headaches mimicking those of a ruptured intracranial aneurysm, with or without concomitant neurologic signs and symptoms, in the context of prolonged but reversible vasoconstriction of cerebral arteries [Chen et al, 2010;Mehdi and Hajj-Ali, 2014]. Diagnostic criteria proposed by Calabrese et al [2007] included (1) acute severe headache, often thunderclap, with or without neurologic signs and symptoms;…”