CENTRAL-NERVOUS-SYSTEM VASCULITIS

Vasculitis is inflammation of blood vessel walls, which produces dysfu nction in both the peripheral and central nervous system (CNS). Cerebr al ischemia is the major cause for neurological manifestations of CNS vasculitis. Unfortunately, a universally accepted classification of va sculitis has not emerged. Vasculitis affecting the CNS alone is referr ed to as primary angiitis of the CNS; secondary vasculitis occurs in a ssociation with a variety of conditions, including infections, drug ab use, lymphoproliferative disease and connective tissue diseases. The p athogenesis of vasculitis includes different immunological mechanisms. Recently, anti-neutrophil cytoplasmatic antibody (ANCA) has been demo nstrated to play an active role in the immunopathogenesis of the vascu litis. Diagnosis of vasculitis depends on a combination of clinical, r adiographic and pathologic features. A wide spectrum of clinical featu res may occur. The most typical clinical picture of CNS vasculitis is troke, encephalopathy or seizures. Assays for ANCA, serum cytokines, a ntibodies to endothelial cell antigens have been reported to be useful in diagnosing or monitoring the disease activity. The gold standard i n diagnosis is confirmation of vasculitis in a biopsy specimen. Angiog raphy may suggest the diagnosis but no abnormalities are pathognomonic . Ideally, the therapy of each vasculitis would focus on the specific immunologic mechanism causing the disease. Such specific interventions are not yet available. In general the most important approaches induc e global immunosuppression. The goal of therapy, however, is to preven t recurrence of disease. (C) 1998 Elsevier Science B.V.