Abstract

Objective: To develop a method to distinguish reversible cerebral vasoconstriction syndrome (RCVS) from other large/medium-vessel intracranial arteriopathies. Methods: We identified consecutive patients from our institutional databases admitted 2013-2017 with newly diagnosed RCVS (n=30) or non-RCVS arteriopathy (n=80). Admission clinical and imaging features were compared. Multivariate logistic regression modelling was used to develop a discriminatory score. Score validity was tested in a separate cohort of patients with RCVS and its closest mimic, primary angiitis of the central nervous system (PACNS). Additionally, key variables were used to develop a bedside approach to distinguish RCVS from non-RCVS arteriopathies. Results: The RCVS group had significantly more women, vasoconstrictive triggers, thunderclap headaches, normal brain imaging results, and better outcomes. Beta coefficients from the multivariate regression model yielding the best c-statistic (0.989) were used to develop the RCVS 2 score (range, -2 to +10; R ecurrent/single thunderclap headache; C arotid artery involvement; V asoconstrictive trigger; S ex; S ubarachnoid hemorrhage). Score ≥5 had 99% specificity and 90% sensitivity for diagnosing RCVS, and score ≤2 had 100% specificity and 85% sensitivity for excluding RCVS. Scores 3-4 had 86% specificity and 10% sensitivity for diagnosing RCVS. The score showed similar performance to distinguish RCVS from PACNS in the validation cohort. A clinical approach based on recurrent thunderclap headaches, trigger and normal brain scans, or convexity subarachnoid hemorrhage, correctly diagnosed 25 of 37 patients with RCVS 2 scores 3-4 across the derivation and validation cohorts. Conclusion: RCVS can be accurately distinguished from other intracranial arteriopathies upon admission, using widely available clinical and imaging features.

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