Abstract
A 67-year-old female presented with multiple dural arteriovenous fistulas (AVFs) manifesting as dementia rapidly progressing over 2 months. The initial diagnosis was Creutzfeldt-Jakob disease based on the acute clinical course. However, angiography eventually revealed multiple dural AVFs involving the bilateral convexities to the superior sagittal sinus and the right transverse-sigmoid sinus. Endovascular treatment combining arterial and venous embolization in multiple stages proved to be effective, as the hemodynamic pathology improved, and the patient recovered from dementia. The cause of the dementia was thought to be venous hypertension in the deep white matter induced by the dural AVFs. Dural AVFs should be included in the differential diagnosis of rapidly progressive dementia.