PRIMARY ANGIITIS OF THE CENTRAL-NERVOUS-SYSTEM

Purpose. - This review is aimed at presenting classification and diagn osis criteria of isolated central nervous system (CNS) angiitis, and a t proposing guidelines for diagnosis and management of this disease. C urrent knowledge and key points. - Isolated CNS angiitis are rare and most information has been provided by studies of very small series. An giitis can be primitive or Secondary to infectious, neoplastic disease s, or toxics. Clinical manifestations and radiologic abnormalities are not specific. A brain biopsy is therefore often required to confirm t he diagnosis, as numerous non-inflammatory vascular diseases can mimic both clinically and radiologically isolated CNS angiitis. Perspective s and projects. - To help guide the diagnosis and therapeutical manage ment of patients with CNS angiitis, strict classification criteria sho uld be used: 1) rule out the various diseases that can mimic clinical and radiological CNS aspects related to isolated angiitis and differen tiate ''isolated CNS angiitis'' from ''CNS angiitis associated with sy stemic diseases''; 2) search for factors associated with the developme nt of a ''secondary CNS angiitis''; 3) check presumed mechanism at the origin of the cerebral vascular disease: ''angiitis'' versus ''angiop athy''; 4) if the diagnosis of ''primary CNS angiitis'' is still suspe cted, it seems reasonable to perform cerebral and leptomeningeal biops ies. Treatment is still unknown and has to be discussed on a case by c ase basis according to the severity and progression of symptoms. (C) 1 998, Elsevier, Paris.