The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS)

Abstract

Introduction To describe clinical, neuroimaging, and laboratory features of a large cohort of Italian patients with reversible cerebral vasoconstriction syndrome. Methods In the setting of the multicenter Italian Project on Stroke at Young Age (IPSYS), we retrospectively enrolled patients with a diagnosis of definite reversible cerebral vasoconstriction syndrome according to the International Classification of Headache Disorders (ICHD)-3 beta criteria (6.7.3 Headache attributed to reversible cerebral vasoconstriction syndrome, imaging-proven). Clinical manifestations, neuroimaging, treatment, and clinical outcomes were evaluated in all patients. Characteristics of reversible cerebral vasoconstriction syndrome without typical causes (“idiopathic reversible cerebral vasoconstriction syndrome”) were compared with those of reversible cerebral vasoconstriction syndrome related to putative causative factors (“secondary reversible cerebral vasoconstriction syndrome”). Results A total of 102 patients (mean age, 47.2 ± 13.9 years; females, 85 [83.3%]) qualified for the analysis. Thunderclap headache at presentation was reported in 69 (67.6%) patients, and it typically recurred in 42 (60.9%). Compared to reversible cerebral vasoconstriction syndrome cases related to putative etiologic conditions (n = 21 [20.6%]), patients with idiopathic reversible cerebral vasoconstriction syndrome (n = 81 [79.4%]) were significantly older (49.2 ± 13.9 vs. 39.5 ± 11.4 years), had more frequently typical thunderclap headache (77.8% vs. 28.6%) and less frequently neurological complications (epileptic seizures, 11.1% vs. 38.1%; cerebral infarction, 6.1% vs. 33.3%), as well as concomitant reversible brain edema (25.9% vs. 47.6%). Conclusions Clinical manifestations and putative etiologies of reversible cerebral vasoconstriction syndrome in our series are slightly different from those observed in previous cohorts. This variability might be partly related to the coexistence of precipitating conditions with a putative etiologic role on disease occurrence.

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

“…There is little evidence on the risk factors for complications. A previous analysis of our cohort found an association of female sex and migraine with hemorrhagic forms of RCVS [6] and several studies documented more complications in patients with secondary compared to idiopathic RCVS [3,7]. Singhal et al identified use of serotonergic antidepressants prior to RCVS and glucocorticoid treatment after onset of RCVS as risk factors for clinical and radiologic worsening [8].…”

“…In our cohort, RCVS patients more often had complications than in the Japanese [16], Korean [7], and Taiwanese [2] cohorts, and less often than in an Italian [3] and two American cohorts [17,18]. Note: OR (95% CI) and p value from stepwise forward logistic regression including the variables thunderclap headache at onset, sex, migraine, postpartum, anxiety or depression, BP surge, and age.…”

“…Reversible cerebral vasoconstriction syndrome (RCVS) consists of headache and multisegmental cerebral vasoconstriction which is reversible within 3 months. Neurological complications occur in 10%–79% of patients [1–3]. The functional outcome is favorable, with complete recovery in the large majority of patients [4,5].…”

Quantifying Intra-Arterial Verapamil Response as a Diagnostic Tool for Reversible Cerebral Vasoconstriction Syndrome

“…The clinical spectrum of RCVS varies among different populations, and the diagnosis of atypical cases can be challenging. 21 Moreover, the classic "string of beads" appearance of vasoconstriction has been described in only 12%-81% of patients with RCVS. 4 Previous reports have suggested the benefit of calcium channel blockers (nicardipine, verapamil, and nimodipine) and phosphodiesterase inhibitors (milrinone) in diagnosing and treating RCVS (On-line Table 3).…”

Vessel Wall Magnetic Resonance Imaging in Cerebrovascular Diseases

“…RCVS is a monophasic disease with a low recurrence rate and relapses are mostly benign [ 76 ]. Triggers as RCVS postpartum state, pre-eclampsia, recreational drugs, vasoactive substances, antidepressants, blood transfusions can be identified in some patients [ 77 ].…”