Fact-sheet: Reversible cerebral vasoconstriction syndrome
Updated on 10/06/2017 at 4:31 PM
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Definition
Association of thunderclap headache and irregularity of the intracranial arteries, reversible within 1 to 3 months. Also known by other names: benign cerebral vasculitis, reversible acute angiopathy, postpartum angiopathy, Call-Fleming syndrome, central nervous system pseudovasculitis.
Clinical features
Population: Women > men. Mean age: 45 years. Headache: thunderclap onset, maximal intensity from the outset, recurrent painful episodes. Neurological deficit and seizures in case of complication of reversible cerebral vasoconstriction syndrome: intraparenchymal hematoma, TIA, infarction.
Laboratory findings
No abnormality.
Lumbar puncture: normal in 50% of cases;
mild abnormalities: moderate pleocytosis,
protein level remaining < 1 g.
CT
Brain CT most often normal. Complications occur in 30% of cases: subarachnoid hemorrhage (20%), intraparenchymal hematoma (5 to 10%), infarction (5%).
MRI
Assessment of the parenchyma to look for complications.
Irregular caliber of the intracranial arteries with alternating stenoses and dilatations.
Vascular intervention
Irregular caliber of the intracranial arteries with alternating stenoses and dilatations if MRI is inconclusive.
Management
Symptomatic medical treatment.
Prognosis usually good; sequelae are possible in case of complications.
Often resolves within a few months.