Narrative Review: Reversible Cerebral Vasoconstriction Syndromes

2 January 2007 | Volume 146 Issue 1 | Pages 34-44

Reversible cerebral vasoconstriction syndromes (RCVS) comprise a group of diverse conditions, all characterized by reversible multifocal narrowing of the cerebral arteries heralded by sudden (thunderclap), severe headaches with or without associated neurologic deficits. Reversible cerebral vasoconstriction syndromes are clinically important because they affect young persons and can be complicated by ischemic or hemorrhagic strokes. The differential diagnosis of RCVS includes conditions associated with thunderclap headache and conditions that cause irreversible or progressive cerebral artery narrowing, such as intracranial atherosclerosis and cerebral vasculitis. Misdiagnosis as primary cerebral vasculitis and aneurysmal subarachnoid hemorrhage is common because of overlapping clinical and angiographic features. However, unlike these more ominous conditions, RCVS is usually self-limited: Resolution of headaches and vasoconstriction occurs over a period of days to weeks. In this review, we describe our current understanding of RCVS; summarize its key clinical, laboratory, and imaging features; and discuss strategies for diagnostic evaluation and treatment.

Author and Article Information

From the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University and Cleveland Clinic Foundation, Cleveland, Ohio; Mayo Clinic, Scottsdale, Arizona; and Massachusetts General Hospital, Boston, Massachusetts.

Grant Support: Dr. Calabrese is supported by the R.J. Fasenmyer Center for Clinical Immunology. Dr. Singhal is supported by NIH grants R01NS051412 and P50NS051343.

Potential Financial Conflicts of Interest: Consultancies: D.W. Dodick (Allergan, AstraZeneca, GlaxoSmithKline, Merck & Co. Inc., Pfizer Inc., Valeant, Ortho-McNeil, Medtronics Inc., Endo); Honoraria: D.W. Dodick (Allergan, AstraZeneca, GlaxoSmithKline, Merck & Co. Inc., Pfizer Inc., Valeant, Ortho-McNeil, Medtronics Inc., Endo); Grants received: D.W. Dodick (Allergan, Medtronics Inc., Advanced Bionics, St. Jude); Expert testimony: A.B. Singhal.

Requests for Single Reprints: Leonard H. Calabrese, DO, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, 9500 Euclid Avenue, Desk A50, Cleveland, OH 44195; e-mail, Calabrl{at}ccf.org.

Current Author Addresses: Dr. Calabrese: Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, 9500 Euclid Avenue, Desk A50, Cleveland, OH 44195.

Drs. Dodick and Schwedt: Department of Neurology, Mayo Clinic, 13400 East Shea Boulevard, Scottsdale, AZ 85259.

Dr. Singhal: Harvard Medical School, Massachusetts General Hospital Stroke Research Center, 175 Cambridge Street, Suite 300, Boston, MA 02114.

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