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Reversible Cerebral Vasoconstriction Syndrome: Updates and New Perspectives

  • Huma U. Sheikh
  • Paul G. Mathew
Uncommon Headache Syndromes (J Ailani, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Uncommon Headache Syndromes

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is an important cause of headaches that can lead to other neurological complications, including stroke, if not recognized early. Over the past few years, there has been great progress in the recognition of this entity. However, there is still much to be learned about its pathophysiology and optimal treatment strategies. RCVS occurs mostly in middle-aged adults, and there is a female preponderance with an increased incidence during the postpartum period. A consistent, predominating feature is a sudden-onset, severe headache that is frequently recurrent, usually over the span of a week. Less common presentations include seizures or focal neurological symptoms. Important causative factors include vasoactive medications, as well as illicit drugs like marijuana and cocaine. The current underlying pathophysiology is thought to be a disturbance in cerebrovascular tone leading to vasoconstriction. The diagnosis is based on history, physical examination, and cerebrovascular imaging findings that demonstrate multifocal, segmental areas of vasoconstriction in large- and medium-sized arteries. An important criterion for making the diagnosis is the eventual reversibility of symptoms and imaging findings.

Keywords

Reversible cerebral vasoconstriction syndrome Thunderclap headache Pathophysiology Vasoconstriction Cerebrovascular imaging 

Notes

Compliance with Ethics Guidelines

Conflict of Interest

Dr. Huma U. Sheikh and Dr. Paul G. Mathew each declare no potential conflicts of interest relevant to this article.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Brigham and Women’s Hospital, Department of Neurology, John R. Graham Headache CenterHarvard Medical SchoolBostonUSA
  2. 2.Cambridge Health Alliance, Division of NeurologyHarvard Medical SchoolCambridgeUSA

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