Localized isolated angiitis of the central nervous system associated with primary intracerebral lymphoma

Isolated angiitis of the central nervous system (IACNS) is a form of granulomatous vasculitis that is confined to the nervous system. A patient with localized IACNS affecting the left internal carotid and posterior cerebral artery adjacent to a primary intracerebral lymphoma of the midbrain along with associated granulomatous inflammation of the dura, leptomeninges, and ependyma of the aqueduct of Sylvius is described. His course was complicated by many neurologic complications before his death. Prednisone and cyclophosphamide were unable to control his disease. More aggressive therapy may be indicated for patients with lymphoproliferative lesions and associated central nervous system (CNS) vasculitis who fail to respond to conventional therapy.