Q :  What are the long-term outcomes after reversible cerebral vasoconstriction syndrome (RCVS)?

 

A: The recurrence rate of RCVS is low (0.65 and 1.71 per 100 person-years from Taiwanese and French cohort respectively. The relapse is mostly benign. A French study showed that the rate of postpartum RCVS was 9% in patient with history of RCVS.  

 

The recurrence of RCVS ranges from 6 months to 7 years after the first episode of RCVS. A Taiwanese cohort, recurrence occurred at a mean of 41± 27.2 months (median 35) and 78±46 months (median 82) in French cohort. The mean follow-up period was 37.5 months in Taiwanese cohort and 110 months in French cohort.

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The only risk factor of recurrence RCVS in Taiwanese study was a sexually triggered thunderclap headache. In the French study, risk factors included a history of migraine and exercised-triggered thunderclap headache during RCVS. Chen et al suggested “Once new thunder-like headaches occur, thorough investigations must be performed again” and “Clinicians should instruct patients to avoid vasoactive substances upon the diagnosis of RCVS”.

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John et al showed that more than half of the patients continued to have mild to moderate chronic headache years after RCVS resolution. Pain and anxiety/depression are frequent.

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Reference:

1. Boitet R, de Gaalon S, Duflos C, Marin G, Mawet J, Burcin C, Roos C, Fiedler U, Bousser MG, Ducros A. Long-Term Outcomes After Reversible Cerebral Vasoconstriction Syndrome. Stroke. 2020 Feb;51(2):670-673

2. Chen SP, Fuh JL, Lirng JF, Wang YF, Wang SJ. Recurrence of reversible cerebral vasoconstriction syndrome: a long-term follow-up study. Neurology. 2015 Apr 14;84(15):1552-8.

3. John S, Singhal AB, Calabrese L, Uchino K, Hammad T, Tepper S, Stillman M, Mills B, Thankachan T, Hajj-Ali RA. Long-term outcomes after reversible cerebral vasoconstriction syndrome. Cephalalgia. 2016 Apr;36(4):387-94.