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Semin Neurol 2020; 40(03): 294-302
DOI: 10.1055/s-0040-1702942
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

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

William Qubty
1   Department of Neurology, The University of Texas at Austin Dell Medical School, Austin, Texas
,
Samantha Lee Irwin
2   Division of Neurology, Department of Pediatrics, University of California San Francisco, San Francisco, California
,
Christine K. Fox
2   Division of Neurology, Department of Pediatrics, University of California San Francisco, San Francisco, California
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Publikationsdatum:
20. Februar 2020 (online)

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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

Disclosures

Dr. Qubty and Dr. Irwin have no relevant disclosures. Dr. Fox receives support from the National Institutes of Health (KL2TR000143), Pediatric Epilepsy Research Foundation, and the University of California San Francisco Benioff Children's Hospital Pediatric Stroke Research Program to investigate pediatric stroke.