How reversible cerebral vasoconstriction syndrome differs from Primary Angiitis of the Central Nervous System
RCVS is more common in women and comprise a group of related disorders characterized by acute onset of recurrent headache (“thunderclap headache”) associated with reversible vasoconstriction of cerebral arteries. These disorders include benign angiitis of CNS, Call–Fleming syndrome, postpartum angiopathy, drug-induced angiopathy (serotonergic, sympathomimetic, illicit), exertional/sex-associated headaches, and migraine-associated angiopathy. All patients present with an acute thunderclap headache which can be associated with seizures (40%), brain edema (40%), lobar or convexity subarachnoid hemorrhage (20%–35%), or generalized seizures (15%–20%). Some patients present with a reversible posterior leukoencephalopathy syndrome. Laboratory and CSF analysis are typically normal. Angiograms show arterial narrowing of intracerebral arteries of both hemispheres. Repeat angiograms in 4 to 12 weeks show resolution of angiographic abnormalities. Failure to reverse angiographic abnormalities within this time period suggests a diagnosis other than RCVS. Treatment includes calcium channel blockers (nimodipine 60 mg every 6 hours, verapamil 80 mg twice daily, or verapamil SR 180–240 mg daily). Some physicians also give a short course of high-dose prednisone.
Pearl: RCVS can be distinguished from PACNS by acuteness of onset, normal CSF exam, and reversibility of angiographic abnormalities within 1 to 3 months.
