Isolated angiitis of the central nervous system

Isolated angiitis of the central nervous system (IACNS) is an idiopathic vasculitis affecting vessels of the CNS. Historically, this disease has been defined as granulomatous angiitis, or the presence of granulomas on postmortem examination. However, other processes, particularly vasculitis secondary to certain malignancies and viral infections display cerebral granulomata. IACNS is also defined as primary angiitis of the CNS. This disorder is very rare and equally affects both genders at any age. Neurological findings are protean and non-specific, although persisting headache, encephalopathy and multifocal signs suggest the diagnosis. Rigorous evaluations are necessary to exclude any cause of secondary vasculitis of CNS and any disease which may be mistaken for IACNS. Diagnosis depends on a combination of clinical, neuroimaging, and histological features, supported hy CSF abnormalities and absence of laboratory evidence of systemic diseases. The treatment with cyclophosphamide, usually combined with prednisone, has greatly improved the prognosis of this disease in the last 20 years and results in a long-term remission or cure in many patients.