Differentiating reversible cerebral vasoconstriction syndrome with subarachnoid hemorrhage from other causes of subarachnoid hemorrhage

JAMA Neurol. 2013 Oct;70(10):1254-60. doi: 10.1001/jamaneurol.2013.3484.


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.

Design: Retrospective analysis of 3 patient cohorts: patients with RCVS (1998-2009), patients with aSAH (1995-2003), and patients with cSAH (1995-2003).

Setting: Academic hospital and tertiary referral center.

Participants: Consecutive patients with RCVS-SAH (n = 38), aSAH (n = 515), or cSAH (n = 93) whose conditions were diagnosed using standard criteria.

Main outcomes and measures: Multivariate logistic regression analysis was used to identify predictors that differentiate RCVS-SAH from aSAH and cSAH.

Results: Predictors differentiating RCVS-SAH from aSAH were younger age, chronic headache disorder, prior depression, prior chronic obstructive pulmonary disease, lower Hunt-Hess grade, lower Fisher SAH group, higher number of affected arteries, and the presence of bilateral arterial narrowing. Predictors differentiating RCVS-SAH from cSAH were younger age, female sex, prior hypertension, chronic headache disorder, lower Hunt-Hess grade, lower Fisher SAH group, and the presence of bilateral arterial narrowing.

Conclusions and relevance: We identified important clinical and imaging differences between RCVS-SAH, aSAH, and cSAH that may be useful for improving diagnostic accuracy, clinical management, and resource utilization.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Cerebral Angiography
  • Cohort Studies
  • Diagnosis, Differential
  • Female
  • Humans
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Predictive Value of Tests
  • Subarachnoid Hemorrhage / complications*
  • Subarachnoid Hemorrhage / diagnosis*
  • Tomography Scanners, X-Ray Computed
  • Vasospasm, Intracranial / classification
  • Vasospasm, Intracranial / diagnosis*
  • Vasospasm, Intracranial / etiology*