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.
Keywords
reversible cerebral vasoconstriction syndrome - benign angiopathy - Call–Fleming's
syndrome - thunderclap headache - pediatric RCVS
