Isolated angiitis of the central nervous system.

Two adults with isolated angiitis of the central nervous system (CNS) are described and the diagnostic criteria of the disease are evaluated. The diagnosis was based on carotid angiogram showing segmental narrowing (a "beaded" appearance) with or without microaneurysms or arterial obstruction, and on clinical observations excluding other underlying diseases. The clinical picture consisted of progressing or fluctuating symptoms and signs of CNS origin. Although the primary etiology of this rare condition remains unknown, treatment with corticosteroids and cyclophosphamide is rewarding. Thus, isolated angiitis of the CNS is treatable, contrary to some earlier assumptions. Therefore, the early diagnosis of isolated angiitis of the CNS is emphasized.