Reversible Cerebral Vasoconstriction Syndromes: Analysis of 139 Cases

Arch Neurol. 2011 Aug;68(8):1005-12. doi: 10.1001/archneurol.2011.68. Epub 2011 Apr 11.

Abstract

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.

Publication types

  • Comparative Study
  • Multicenter Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Cerebrovascular Disorders / diagnosis*
  • Cerebrovascular Disorders / epidemiology*
  • Cerebrovascular Disorders / therapy
  • Cohort Studies
  • Comorbidity
  • Female
  • Humans
  • Male
  • Middle Aged
  • Retrospective Studies
  • Syndrome
  • Treatment Outcome
  • Vasospasm, Intracranial / diagnosis*
  • Vasospasm, Intracranial / epidemiology*
  • Vasospasm, Intracranial / therapy
  • Young Adult