Reversible Cerebral Vasoconstriction Syndromes

Abstract

Objectives: To compare the clinical, laboratory, and imaging features of patients with reversible cerebral vasoconstriction syndromes evaluated at 2 academic centers, compare subgroups, and investigate treatment effects. Design: Retrospective analysis. Setting: Massachusetts General Hospital (n = 84) or Cleveland Clinic (n = 55). Patients: One hundred thirty-nine patients with reversible cerebral vasoconstriction syndromes. Main Outcome Measures: Clinical, laboratory, and imaging features; treatment; and outcomes. Results: The mean age was 42.5 years, and 81% were women. Onset with thunderclap headache was documented in 85% and 43% developed neurological deficits. Prior migraine was documented in 40%, vasoconstrictive drug exposure in 42%, and recent pregnancy in 9%. Admission computed tomography or magnetic resonance imaging was normal in 55%; however, 81% ultimately developed brain lesions including infarcts (39%), convexity subarachnoid hemorrhage (34%), lobar hemorrhage (20%), and brain edema (38%). Cerebral angiographic abnormalities typically normalized within 2 months. Nearly 90% had good clinical outcome; 9% developed severe deficits; and 2% died. In the combined cohort, calcium channel blocker therapy and symptomatic therapy alone showed no significant effect on outcome; however, glucocorticoid therapy showed a trend for poor outcome (P=.08). Subgroup comparisons based on prior headache status and identified triggers (vasoconstrictive drugs, pregnancy, other) showed no major differences. Conclusion: Patients with reversible cerebral vasoconstriction syndromes have a unique set of clinical imaging features, with no significant differences between subgroups. Prospective studies are warranted to determine the effects of empirical treatment with calcium channel blockers and glucocorticoids.

Hemorrhagic Reversible Cerebral Vasoconstriction Syndrome

“…In a previous study, we found no major differences in RCVS subgroups defined by risk factors and triggers. 4 These data suggest that the phenotype of RCVS is relatively homogenous regardless of lesion type or precipitant.…”

“…The clinical and brain-imaging features of the first 159 patients have been published. 1,4 We were careful to exclude patients with primary angiitis of the central nervous system, a close mimic of RCVS, based on published criteria. 1 Serial cerebral angiography confirmed reversibility in 128 patients, and 30 patients developed sudden, severe headaches, segmental arterial narrowing on cerebral angiography, and had a self-limited clinical course in the absence of immunosuppressive treatment and no recurrences during follow-up.…”

“…Limitations of our analysis include the inconsistent time of imaging (which was driven by clinical indications), and hence, the subacute findings may be biased toward symptomatic lesions such as new infarctions. Lesion development (especially infarction) may be influenced by iatrogenic factors, such as glucocorticoid therapy, 4 calcium-channel blocker or vasodilator treatment, or angiographic complications, 18 which were not considered in this study.…”

“…[1][2][3][4][5] Approximately one third to half develop intracerebral hemorrhage (ICH), convexal subarachnoid hemorrhage (cSAH), ischemic stroke, and reversible brain edema (posterior reversible leukoencephalopathy syndrome or posterior reversible encephalopathy syndrome [PRES]), either alone or in combination. [6][7][8][9] The pathophysiology of this syndrome is not known.…”

“…In a previous study, we showed that there were no significant differences between RCVS subgroups as defined by presumed risk factors and triggers, such as previous migraine and vasoconstrictive drugs. 4 The aim of this study was to understand the mechanisms (the role of risk factors, triggers, and angiographic evolution) in hemorrhagic and nonhemorrhagic RCVS.…”