RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome

Neurology. 2019 Feb 12;92(7):e639-e647. doi: 10.1212/WNL.0000000000006917. Epub 2019 Jan 11.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Cerebral Angiography
  • Cerebrovascular Disorders / diagnosis
  • Computed Tomography Angiography
  • Diagnosis, Differential
  • Female
  • Headache Disorders, Primary / etiology
  • Humans
  • Intracranial Arterial Diseases / complications
  • Intracranial Arterial Diseases / diagnosis*
  • Intracranial Arteriosclerosis / diagnosis*
  • Male
  • Middle Aged
  • Moyamoya Disease / diagnosis*
  • Puerperal Disorders / diagnosis*
  • Retrospective Studies
  • Sex Factors
  • Subarachnoid Hemorrhage / diagnosis
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasoconstriction

Supplementary concepts

  • Primary angiitis of the central nervous system