We report four fatal cases of fulminant reversible cerebral vasoconstriction syndrome, all initially diagnosed as primary central nervous system vasculitis and treated with corticosteroids. Although reversible cerebral vasoconstriction syndrome is usually self-limiting without permanent neurologic deficits, rarely it can be fatal and worse outcomes have been associated with corticosteroid treatment.
Keywords: Corticosteroids; Headache; Primary angiitis of the central nervous system; Reversible cerebral vasoconstriction syndrome; Thunderclap headache; Vasculitis.
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