Reversible Cerebral Vasoconstriction Syndrome

KEY FACTS

TERMINOLOGY

• Reversible cerebral vasoconstriction syndrome, a.k.a. CallFleming 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)

(Left) Axial NECT in a female

patient with acute onset of

severe headache shows

limited cortical subarachnoid

hemorrhage (SAH) in the sulci

of the frontal lobes ſt. DSA

was performed to exclude a

vascular lesion and showed

multifocal arterial narrowings

(not shown) related to RCVS.

(Right) Axial DWI trace shows

multiple foci of hyperintensity

﬇ related to acute ischemia

in this patient with RCVS who

presented with an acute onset

of a "thunderclap" headache.

Ischemia is a common

complication of RCVS.

(Left) Anteroposterior right

vertebral artery DSA shows

diffuse luminal irregularity

and focal stenoses involving

the basilar artery and

posterior cerebral and superior

cerebellar arteries ﬈. Similar

changes were seen in the

anterior circulation (not

shown). The patient was

treated with IA verapamil over

the next 10 days. (Right) DSA

repeated 2 weeks later shows

significant interval resolution

of the posterior circulation

vasospasm. There are a few

foci ﬈ of mild residual

stenoses still evident.

Brain: Pathology-Based Diagnoses: Malformations,

Trauma, and Stroke

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