Reversible cerebral vasoconstriction syndromes: analysis of 139 cases.


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.

DOI: 10.1001/archneurol.2011.68

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@article{Singhal2011ReversibleCV, title={Reversible cerebral vasoconstriction syndromes: analysis of 139 cases.}, author={Aneesh Bhim Singhal and Rula A. Hajj-Ali and Mehmet Akif Topçuoğlu and Joshua W Fok and James Frank Bena and Donsheng Yang and Leonard H. Calabrese}, journal={Archives of neurology}, year={2011}, volume={68 8}, pages={1005-12} }