Granulomatous angiitis of the central nervous system

Granulomatous angiitis of the nervous system is a rare necrotizing vasculitis that is largely confined to the central nervous system. The case of a 43-year-old patient is presented and the clinical presentation, diagnostic difficulties and treatment are discussed. Patients present in their fourth decade with diffuse and focal neurological symptoms and signs. No single test is diagnostic; however, cerebrospinal fluid lymphocytosis and raised protein levels, changes on cerebral angiography, and leptomeningeal biopsy may confirm the diagnosis. Although the administration of corticosteroid agents has prolonged the survival of the patient, a progression of the disease has been the rule. The administration of corticosteroid and cyclophosphamide agents on alternate days may lead to clinical remission of the disease.