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.

Reversible cerebral vasoconstriction syndromes (RCVS) and primary angiitis of the central nervous system (PACNS) are invariably considered in the differential diagnosis of new cerebral arteriopathies. However, prompt and accurate diagnosis remains challenging. Here we compared the features of 159 RCVS to 47 PACNS patients and developed criteria for prompt bedside diagnosis. Recurrent thunderclap headache (TCH), and single TCH combined with either normal neuroimaging, border zone infarcts, or vasogenic edema, have 100% positive predictive value for diagnosing RCVS or RCVS-spectrum disorders. In patients without TCH and positive angiography, neuroimaging can discriminate RCVS (no lesion) from PACNS (deep/brainstem infarcts). Ann Neurol 2016;79:882-894.

IMPORTANCE Reversible cerebral vasoconstriction syndrome (RCVS) is a clinical-angiographic syndrome characterized by recurrent thunderclap headaches and reversible segmental multifocal cerebral artery narrowing. More than 30% of patients with RCVS develop subarachnoid hemorrhage (SAH). Patients with RCVS with SAH (RCVS-SAH) are often misdiagnosed as having potentially ominous conditions such as aneurysmal SAH (aSAH) or cryptogenic "angiogram-negative" SAH (cSAH) owing to overlapping clinical and imaging features. OBJECTIVE To identify predictors that can distinguish RCVS-SAH from aSAH and cSAH at the time of clinical presentation.

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Background: Reversible cerebral vasoconstriction syndrome (RCVS) is a self-limited entity with usually benign outcome. Over 30% RCVS patients develop subarachnoid hemorrhage (SAH). We aimed to identify features that differentiate RCVS-SAH from more ominous causes of SAH, i.e. aneurysmal SAH (aSAH) and cryptogenic ‘angio-negative’ SAH (cSAH). Methods: We compared the clinical-imaging features of 38 consecutive RCVS-SAH patients, to 515 aSAH and 93 cSAH patients consecutively admitted to Massachusetts General Hospital. Results: As compared to aSAH and cSAH, the RCVS-SAH group was significantly younger, more women, and higher frequency of migraine, depression, chronic obstructive pulmonary disease (COPD), alcohol and drug exposure, and prior antidepressant use. The distribution of Hunt-Hess (HH) grade and Fisher group were different between groups, with median values highest in the aSAH group. The RCVS-SAH group had more hypodense lesions on 1st head CT and earlier, more severe and widespread vasoconstriction on cerebral angiography. Discharge mRS scores were lowest in the RCVS-SAH group. To avoid overfitting, multivariate logistic regression (Firth’s method) was performed using separate models for clinical and radiological variables given small "N". Predictors of RCVS-SAH vs. aSAH [model 1]: age (O.R. 0.9, 95% C.I. 0.9-0.96), prior headache disorder (O.R. 9.3, 95% C.I. 3.9-22.4), depression (O.R. 5.6, 95% C.I. 1.8-17.6), and COPD (O.R. 7.6, 95% C.I. 2.9-20.1), and [model 2]: HH grade (O.R. 0.4, 95% C.I. 0.2-0.7), Fisher group (O.R. 0.2, 95% C.I. 0.07-0.4), and the number of constricted arteries (O.R. 1.6, 95% C.I. 1.4-1.9). Predictors of RCVS-SAH vs. cSAH [model 1]: age (O.R. 0.9, 95%C.I. 0.9-0.97), prior headache (O.R. 10.3, 95% C.I. 4.3-24.9), depression (O.R. 6.9, 95% C.I. 2.1-22.4), and alcohol use (O.R. 5.1, 95% C.I. 2.0-12.9), and [model 2]: Fisher group (O.R. 0.01, 95% C.I. 0.0-0.6), vasospasm severity (O.R. 9.1, 95% C.I. 1.4-57.2), and the number of constricted arteries (O.R. 2.0, 95% C.I. 1.2-3.1). Conclusion: Several clinical-imaging features distinguish RCVS-SAH from aSAH and cSAH. These data should prove useful to improve the diagnostic accuracy, management, and resource utilization in patients with SAH.