Type of headache at onset and risk for complications in reversible cerebral vasoconstriction syndrome

Abstract

Background In a recent Italian study, 30% of patients with reversible cerebral vasoconstriction syndrome (RCVS) presented without thunderclap headache (TCH), and tended to present more severe forms of RCVS than patients with TCH. We aimed to analyze the risk for complications of RCVS in patients with and without TCH at onset. Methods In a pooled cohort of 345 French patients with RCVS, we compared patients with and without TCH at onset regarding rates of neurological complications, and the functional outcome at 3 months. Results As compared to the 281 patients with TCH at onset, the 64 patients without TCH had a higher risk for any neurological complication (61% vs. 24%, OR 4.9, 95% CI 2.8–8.7, p < 0.001). The association was strongest for cervical artery dissections (28% vs. 5%, OR 8.1, 95% CI 3.7–17.6, p < 0.001), followed by posterior reversible encephalopathy syndrome (17% vs. 3%, OR 7.1, 95% CI 2.7–18.4, p < 0.001), seizures (9% vs. 2.5%, OR 4.1, 95% CI 1.3–12.5, p = 0.019), and subarachnoid hemorrhage (41% vs. 16%, OR 3.5, 95% CI 1.9–6.3, p < 0.001). In multivariable analysis, the risk for any neurological complication remained significantly elevated in the absence of TCH (OR 3.5, 95% CI 1.8–6.8, p < 0.001). The functional outcome was equal in both groups, with a modified Rankin scale score of 0–1 in ≥90% of patients. Conclusions Absence of TCH at onset might predict a higher risk of complications in RCVS. Our results warrant further multicentric studies to prove this finding.

Pathophysiology of reversible cerebral vasoconstriction syndrome

“…Overall, vasoactive substances and post-partum state are the most common secondary causes of RCVS. In previously reported large series, RCVS is predominately idiopathic in Asian populations while the opposite is observed in Europeans or Americans [ 2 , 13 – 20 ]. Patients with RCVS are predominantly female with age around 40–55, while male patients are 10 years younger than the female patients [ 2 , 15 , 17 ].…”

“…Accordingly, RCVS has been seen by physicians across disciplines but only until the past two decades has it been recognized as a distinct clinic-radiological syndrome. Large case series of RCVS have been reported in Asia, Europe and America [2,[13][14][15][16][17][18][19][20]]. Yet, the exact epidemiology of RCVS remains unclear.…”

Reversible cerebral vasoconstriction syndrome: A review of pathogenesis, clinical presentation, and treatment

“…RCVS without TCH is reported in 10-30%, 51 perhaps due to limited recall in patients with encephalopathy or seizures. Pregnant women have a lower rate of TCH.…”

“…Pregnant women have a lower rate of TCH. 51 The pain is extreme, but subsides within minutes to hours, only to recur periodically over the next several days. A prospective study found an average of 4.5 TCH that recurred over 7.4 (range: 1-26) days.…”

“…Post-partum women, 80,81 patients without TCH, and those with early infarcts or who are inadvertently treated with triptans or glucocorticoids, have the highest risk for complications. 20,24,51 Wide BP fluctuations are common initially, perhaps from autoregulation or the stress of severe headache. It is advisable to avoid hypotension in the setting of severe vasoconstriction.…”