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Narrative Review: Reversible Cerebral Vasoconstriction Syndromes

Leonard H. Calabrese, DO; David W. Dodick, MD; Todd J. Schwedt, MD; and Aneesh B. Singhal, MD
[+] Article and Author 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@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.


Ann Intern Med. 2007;146(1):34-44. doi:10.7326/0003-4819-146-1-200701020-00007
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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.

Figures

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Figure 1.
Neuroimaging findings in a 46-year-old man with reversible cerebral vasoconstriction syndrome.

The patient, who had a history of migraine without aura, hypertension, hyperlipidemia, and cannabis abuse, developed a severe postcoital “thunderclap” headache. Severe headaches recurred, and on day 3, he developed cortical blindness and mild left hemiparesis. Computed tomography angiography obtained at admission showed multifocal segmental stenosis (“beading”) of the bilateral middle cerebral arteries (A) and the basilar, posterior cerebral, and superior cerebellar arteries (B). These abnormalities were also present on brain magnetic resonance angiography (C). Diffusion-weighted magnetic resonance imaging (D) and apparent diffusion coefficient maps (E) showed symmetrical lesions in the bilateral occipital lobes consistent with ischemic stroke. In addition, brain magnetic resonance imaging showed small infarctions in the bilateral cerebellar hemispheres and in the right frontal lobe (not shown). Serologic tests and the results of 2 cerebrospinal fluid examinations showed no evidence for vasculitis or subarachnoid hemorrhage. The patient was treated with analgesics and verapamil. His deficits resolved completely over a period of 3 weeks, and follow-up magnetic angiography (F) showed resolution of the cerebral arterial vasoconstriction.

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Grahic Jump Location
Figure 2.
Cerebral angiography of a patient with reversible cerebral vasoconstriction syndrome at diagnosis (left) and after 1 month of calcium-channel blocker therapy (right).

Note the multiple areas of stenosis (white arrows) and dilatation in multiple vessels (blackarrows) and their resolution after treatment. Reprinted with permission from reference 36: Hajj-Ali RA, Furlan A, Abou-Chebel A, Calabrese LH. Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. Arthritis Rheum. 2002;47:662-9.

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Refining Reversible Cerebral Vasoconstriction Syndromes
Posted on March 5, 2007
Shuu-Jiun Wang
Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan
Conflict of Interest: None Declared

To the Editor: Calabrese and colleagues (1) recently published a narrative review on reversible cerebral vasoconstriction syndromes (RCVS), which summarized clinical, laboratory and imaging features, and discussed strategies for diagnosis and treatment. This comprehensive work provides guidance for both rheumatologists and neurologists; however, we have some concerns and would like to update and take some specific issue with the information raised in this article.

The authors exemplified myriads of eponymic labels of similar syndromes and proposed RCVS as a new collective terminology. One of the syndromes which the authors chose to include in their proposed grouping is benign angiopathy of the central nervous system (CNS). It has been adopted as a distinct diagnostic entity (Code 6.7.3) in the International Classification of Headache Disorders, 2nd edition (ICHD-2), 2004 (2). For those without cerebral vasoconstrictions, the ICHD-2 renders another diagnostic entity as ¡§primary thunderclap headache (code 4.6). We recently field-tested and published our findings on these two diagnostic criteria using a large cohort of idiopathic recurrent thunderclap headache patients and demonstrated that the ICHD-2 criteria were indeed valid except for the duration criterion (3). Since structured criteria have been developed in the ICHD-2, we suggest applying these diagnostic entities and their criteria with modifications if needed.

Secondly, the authors quoted a review (4) which disclosed that stroke was found in up to 54% of patients with RCVS; however, it must be pointed out that the patients in the review (4) were collected from 114 selected articles, which might have yielded a high reporting bias. In our prospective study of patients with benign angiopathy of the CNS (3), the stroke risk was, by comparison, only 14 % (3/22). Thirdly, as for treatment strategies, we would like to provide a different view. Since recurrent thunderclap headache patients usually suffer from intense paroxysmal headaches for up to 2 weeks to one month, we suggest a calcium channel blocker, such as nimodipine to be used not only for those with cerebral vasoconstriction but also for those without to abort further unbearable attacks. Although no controlled trials have been done, we previously reported that nimodipine was shown to be effective with good tolerance (3,5). Fourthly, after studying consecutive patients, we believe conventional angiography and CSF studies should be reserved for those with a low pretest probability. A careful and detailed medical history along with MRI, MRA and MRV studies are sufficient to diagnose most patients with recurrent thunderclap headache.

References:

1. Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative Review: reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146:34-44. [PMID: 17200220]

2. Headache Classification Subcommittee of the International Headache Society. The International classification of headache disorders, 2nd ed. Cephalalgia 2004;24(Suppl 1):1-160.

3. Chen SP, Fuh JL, Lirng JF, Chang FC, Wang SJ. Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology. 2006;67:2164-9. [PMID: 17190937]

4. Singhal AB. Cerebral vasoconstriction without subarachonoid blood: associated conditions, clinical and neuroimaging characteristics. Ann Neurol. 2002:S:59-60

5. Lu SR, Liao YC, Fuh JL, Lirng JF, Wang SJ. Nimodipine for treatment of primary thunderclap headache. Neurology. 2004;62:1414-6. [PMID: 15111686]

Conflict of Interest:

None declared

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