Angiitis of the central nervous system

Angiitis of the central nervous system is a rare disease which may result f rom numerous causes responsible for the presence of inflammatory lesions of the vascular wall. These inflammatory lesions may sometimes be associated with necrosis. Cerebral vessels of all sizes may be involved. The clinical presentation is highly variable, with focal to diffuse manifestations and a cute to chronic evolution. Angiography is the cornerstone diagnostic proced ure, showing multiple segmental stenoses of the cerebral arteries sometimes separated by fusiform dilatations. Although suggestive, this angiographica l pattern is not unequivocal and other causes must be carefully ruled out. Only cerebral and/or leptomeningeal biopsy can provide a definite diagnosis of cerebral angiitis but this invasive diagnostic procedure is not perform ed in the majority of cases. Among the numerous causes of cerebral angiitis , one can individualize infectious diseases, primary systemic angiitis with cerebral involvement, angiitis secondary to various systemic diseases and other miscellaneous causes such as drug abuse or neoplasm.