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Reviews:
Leonard H. Calabrese, David W. Dodick, Todd J. Schwedt, and Aneesh B. Singhal
Narrative Review: Reversible Cerebral Vasoconstriction Syndromes
Ann Intern Med 2007; 146: 34-44 [Abstract] [Full text] [PDF]
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[Read Rapid Response] Refining Reversible Cerebral Vasoconstriction Syndromes
Shuu-Jiun Wang, Jong-Ling Fuh, Shih-Pin Chen   (5 March 2007)

Refining Reversible Cerebral Vasoconstriction Syndromes 5 March 2007
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Shuu-Jiun Wang,
MD
Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan,
Jong-Ling Fuh, Shih-Pin Chen

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Re: Refining Reversible Cerebral Vasoconstriction Syndromes

sjwang{at}vghtpe.gov.tw Shuu-Jiun Wang, et al.

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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