Reversible cerebral vasoconstriction syndrome
Reversible cerebral vasoconstriction syndrome (RCVS) is recurrent thunderclap headache with multifocal constriction of intracranial arteries that normalizes within 3 months, usually triggered by vasoactive drugs or the puerperium, and it is the second most frequent cause of thunderclap headache after subarachnoid hemorrhage. Diagnosis follows noncontrast CT, lumbar puncture, and MRA or CTA; treatment is removal of the trigger, blood pressure control, and verapamil, with glucocorticoids and triptans avoided.
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What this article covers
- Overview
Definition: reversible cerebral vasoconstriction syndrome (RCVS) is recurrent thunderclap headache with multifocal constriction of intracranial vessels that normalizes within 3 months of onset.
- Epidemiology
Frequency among thunderclap headaches: second only to subarachnoid hemorrhage, which accounts for nearly 25% of thunderclap presentations.
- Risk factors
Vasoactive drug exposure, the leading predisposing category: sympathomimetics (amphetamines, pseudoephedrine, phenylpropanolamine) antidepressants (SSRIs, SNRIs, monoamine oxidase inhibitors)…
- Pathophysiology
Transient, multifocal, segmental constriction of intracranial arteries is the defining lesion, seen as multifocal distal arterial narrowing that resolves within 3 months.
- Clinical features
Recurrent thunderclap headaches: severe headaches that reach maximum intensity within 1 minute and recur; this pattern plus multifocal reversible vasoconstriction on vessel imaging is the defining syndrome.
- Differential diagnosis
Subarachnoid hemorrhage: the most common and most dangerous cause of thunderclap headache; blood on noncontrast CT, or a CSF erythrocyte count above 10,000/microL with xanthochromia, and an aneurysm on vessel imaging.
- Diagnosis
The diagnosis is established by recurrent thunderclap headache plus multifocal segmental constriction of intracranial arteries on MRA or CTA that normalizes within 3 months: typically with a normal…
- Management
First, remove the trigger: stop sympathomimetics, serotonergic antidepressants, triptans, ergots, nicotine, cannabis, and illicit drugs, and investigate a catecholamine-secreting tumor when suggested.
- Complications
Ischemic stroke from vasoconstriction, seen as areas of parenchymal ischemia on MRI.
- Prognosis
Vasoconstriction normalizes within 3 months by definition, which is why repeat MRA or CTA at 12 weeks is the end point of treatment.
- Prevention
Avoid re-exposure to the identified trigger, particularly sympathomimetics (including pseudoephedrine), serotonergic antidepressants, triptans, ergots, cocaine, amphetamines, and cannabis.
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