Clinical Analysis of Isolated Angiitis of the Central Nervous System: A Report of 11 Cases

Isolated angiitis of the central nervous system may occur more frequently, and with a more favorable outcome, than has previously been described. During the past 10 years, we diagnosed this condition in 11 patients ranging in age from 23 to 38 years whose initial presenting symptoms included severe headache, transient focal neurologic deficits, or seizures. The diagnosis was established in all cases by characteristic angiographic findings. All patients were initially treated with high-dose steroids; one patient later required the addition of an immunosuppressive agent. All patients responded initially to the steroids, and 10 were in clinical remission while receiving either a small dose of steroids or no medication after a mean follow-up of 33 months. One patient died 9 months after diagnosis, presumably of an intracerebral hemorrhage. This experience suggests that the diagnosis of isolated angiitis of the central nervous system should be considered in any young person complaining of persistent headache, transient focal neurologic deficits, or seizures. Angiography usually yields characteristic diagnostic findings. Treatment should consist of high-dose steroids, with the addition of immunosuppressive agents only in cases where steroids are ineffective.