[Vasculitis of the central nervous system]

Vasculitis of the central nervous system (CNS) is a rare disease characterized inflammation and/or sclerosis of the vascular wall, usually due to an immunological mechanism. The positive diagnosis of CNS vasculitis is difficult owing to its protean clinical manifestations, to its variable course and to the nonspecificity of CT and MRI images. Angiography itself may be of no avail since the segmental narrowing images, although suggestive, are neither constant nor pathognomonic. The causes of CNS vasculitis are very numerous. The aetiological diagnosis is easy when the causal systemic disease is already known or when an inflammatory syndrome and other localizations are present, but it is difficult in those exceptional cases where the vasculitis is limited to the CNS. Biopsy of the cortex and leptomeninges may them be required. Treatment varies according to the cause; it rests essentially on corticosteroid therapy, sometimes combined with cyclophosphamide.