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The primary angiitis of the central nervous system (PACNS) is an entity with a very low incidence and prevalence. It affects small and medium sized arteries of the brain parenchyma, spinal cord and leptomeniges resulting in CNS dysfunction. It is defined by inflammation of the cerebral vasculature without angiitis of other organ. Its clinical manifestations are very heterogeneous and make clinical diagnosis difficult. In most cases, a brain biopsy is required. Only the clinical suspicion anddoi:10.18203/2320-6012.ijrms20150280 fatcat:zivvej2bz5h73icnd7yhfz6r64