Reversible Cerebral Vasoconstriction Syndrome

KEY FACTS

Terminology

• Reversible cerebral vasoconstriction syndrome, a.k.a. Call-Fleming syndrome

• Group of disorders characterized by

  • Reversible, multifocal cerebral artery vasoconstrictions

  • Severe headaches ± focal neurological deficits

Imaging

• Acute/recurrent headaches with vasculitic pattern (DSA)

• DSA = crucial for diagnosis (100% sensitive)

  • Involves large, medium-sized arteries

  • Diffuse, multifocal, segmental narrowing

  • Sometimes “string of beads” or “sausage strings”

• NECT often negative

  • Small cortical subarachnoid hemorrhages (20%) ± parenchymal hemorrhage

• CTA/MRA: May be normal if subtle changes (10%)

  • Diffuse segmental arterial constriction in 90%

• Transcranial Doppler: ↑ arterial velocities in MCA, ICA, & ACA

Pathology

• Thought to represent transient disturbance in control of cerebral vascular tone → vasoconstriction → ischemia, stroke, death

• Spontaneous (1/3 of cases) or precipitated by

  • Postpartum state

  • Exposure to vasoactive substances

• Can be associated with posterior reversible encephalopathy syndrome (PRES)

Clinical Issues

• Symptoms: Severe, acute “thunderclap” headache

  • Often recurrent (95%)

  • Ischemia/stroke (visual disturbance, aphasia, hemiparesis)

• Treatment: Discontinuation of vasoactive medications; vasodilators (e.g., Ca++ channel blockers)

TERMINOLOGY