An unusual form of vasculitis involving the dural sinuses and extracranial arteries — A case report

Abstract

Cerebral venous thrombosis (CVT) associated with vasculitis has been described only in the context of small vessel angiitis so far. We report on a 30-year-old woman who developed CVT which was followed by progressive occlusion of large extracranial arteries. Cerebrospinal fluid (CSF) showed evidence for inflammation with pleocytosis and positive oligoclonal bands. Angiography revealed no abnormalities of the aortic arch, but three remaining criteria for Takayasu arteritis were fulfilled. Antiphospholipid antibodies were elevated or within borderline values, possibly indicating the beginning of lupus erythematosus. Because of additional protein S deficiency and increased factor VIII activity, the patient was orally anticoagulated. Immunosuppressive therapy was started with azathioprine. This was followed by a normalization of cell count in CSF. Immunosuppression was changed to methotrexate after acute progression of the occlusive disease. As far as we know this is the first case report with a combination of sinus thrombosis and large vessel arteritis.