Review on the Diagnosis and Treatment of Reversible Cerebral Vasoconstriction Syndrome in Children and Adolescents

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical–radiologic diagnosis that affects children and adolescents, but it is much more frequently reported in adults. Clinically, patients present with severe and commonly recurrent thunderclap headaches. Typical precipitating triggers include vasoactive substances, serotonergic agents, and the postpartum period. There may be associated neurologic complications at presentation or in the weeks following, such as convexity subarachnoid hemorrhage, stroke, cerebral edema, cervical artery dissection (CeAD), and seizures. Angiographically, the cerebral arteries demonstrate segmental vasoconstriction and dilation, although imaging early in the clinical course may be normal. Work-up is performed to exclude intracranial disorders such as vasculitis, subarachnoid hemorrhage due to ruptured aneurysm, meningitis, and intracranial venous sinus thrombosis. Within 1 month of initial symptom onset, clinical symptoms such as severe headache have ceased, and within 3 months, the cerebral vasoconstriction is much improved or resolved. Management involves avoidance of precipitating triggers and potentially short-term pharmacotherapy with calcium channel blockers for patients with associated neurologic complications. Steroids are not recommended and may worsen the clinical outcome. Prognosis is excellent in the large majority of patients, and only 5% of patients experience a recurrence of RCVS.

Reversible Cerebral Vasoconstriction Syndrome in a Previously Healthy Child

“…2,3,5,6 It has also been associated with sympathomimetic medication and immunosuppression. 1 In our case, though, no trigger was identified.…”

Posterior Reversible Encephalopathy Syndrome in Guillain-Barré Syndrome: Just a Problem of Immunoglobulins? Controversy From Two Atypical Case Reports

“…Reversible cerebral vasoconstriction syndrome (RCVS) is a group of clinical entities characterized by prolonged, reversible vasoconstriction of the cerebral arteries that resolve within 3 months, associated with a rapid or gradual onset of severe headache, and complicated by other neurological symptoms (stroke, hemorrhage, encephalopathy, concomitant PRES, and seizures) in up to one-third of patients ( 2 ).…”

Reversible Cerebral Vasoconstriction Syndrome Presenting with Thunderclap Headache in a Child

“…A large adult case series showed that RCVS commonly affects middle-aged women [5,6]. In contrast, a review of pediatric RCVS patients reported a male predominance [2,7]. Brain computed tomographic angiography or MRA is useful for diagnosing RCVS.…”

“…Brain computed tomographic angiography or MRA is useful for diagnosing RCVS. However, signs of vasoconstriction can be noticed 7 to 14 days after the onset of the headache [2,7]. Headache can precede the onset of brain angiography findings showing vasoconstriction, which causes difficulties in accurate diagnosis [2].…”

“…The most important treatment for RCVS is supportive care such as hydration, bed rest, analgesics, blood pressure control, and avoiding triggering factors [7]. Calcium channel blockers are the most commonly used medications for vasodilation in the acute phase of RCVS [7].…”