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.

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”.

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.

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.