Eva A Rocha1, M Akif Topcuoglu1, Gisele S Silva1, Aneesh B Singhal2. 1. From the Department of Neurology (E.A.R., A.B.S.), Massachusetts General Hospital, Boston; Department of Neurology (E.A.R., G.S.S.), Universidade Federal de São Paulo, Brazil; Hospital Israelita Albert Einstein (G.S.S.), São Paulo, Brazil; and Department of Neurology (M.A.T.), Hacettepe University, Ankara, Turkey. 2. From the Department of Neurology (E.A.R., A.B.S.), Massachusetts General Hospital, Boston; Department of Neurology (E.A.R., G.S.S.), Universidade Federal de São Paulo, Brazil; Hospital Israelita Albert Einstein (G.S.S.), São Paulo, Brazil; and Department of Neurology (M.A.T.), Hacettepe University, Ankara, Turkey. asinghal@partners.org.
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 in 2013-2017 with newly diagnosed RCVS (n = 30) or non-RCVS arteriopathy (n = 80). Admission clinical and imaging features were compared. Multivariate logistic regression modeling 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 CNS (PACNS). In addition, 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 RCVS2 score (range -2 to +10; recurrent/single thunderclap headache; carotid artery involvement; vasoconstrictive trigger; sex; subarachnoid 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 RCVS2 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. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that the RCVS2 score accurately distinguishes patients with RCVS from those with other intracranial arteriopathies.
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 in 2013-2017 with newly diagnosed RCVS (n = 30) or non-RCVS arteriopathy (n = 80). Admission clinical and imaging features were compared. Multivariate logistic regression modeling 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 CNS (PACNS). In addition, 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 RCVS2 score (range -2 to +10; recurrent/single thunderclap headache; carotid artery involvement; vasoconstrictive trigger; sex; subarachnoid 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 RCVS2 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. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that the RCVS2 score accurately distinguishes patients with RCVS from those with other intracranial arteriopathies.
Authors: J M Sequeiros; J A Roa; R P Sabotin; S Dandapat; S Ortega-Gutierrez; E C Leira; C P Derdeyn; G Bathla; D M Hasan; E A Samaniego Journal: AJNR Am J Neuroradiol Date: 2020-09-17 Impact factor: 3.825
Authors: Caleigh Mandel-Brehm; Hanna Retallack; Giselle M Knudsen; Alex Yamana; Rula A Hajj-Ali; Leonard H Calabrese; Tarik Tihan; Hannah A Sample; Kelsey C Zorn; Mark P Gorman; Jennifer Madan Cohen; Antoine G Sreih; Jacqueline F Marcus; S Andrew Josephson; Vanja C Douglas; Jeffrey M Gelfand; Michael R Wilson; Joseph L DeRisi Journal: Neurology Date: 2019-07-03 Impact factor: 9.910