Progressive Neurological Decline with Deep Bilateral Imaging Changes: A Protean Presentation of Dural Arteriovenous Fistulae.

Colorado, Rene A; Matiello, Marcelo; Yang, Hyun-Sik; Rabinov, James D; Patel, Aman; Hirsch, Joshua A; Chavali, Ram; Leslie-Mazwi, Thabele M

Intracranial dural arteriovenous fistulae (DAVF) within the deep cerebral vasculature are diagnostically challenging because of their variable clinical presentation and typical bilateral neuroimaging findings mimicking inflammatory, infectious, and metabolic processes. Increasingly, reports have emerged highlighting the diagnostic and treatment challenges of these lesions and their associated high morbidity and rapid clinical deterioration when untreated. We describe here a case series of 4 patients with deep cerebral DAVF who presented with impaired arousal or memory and behavioral changes. In all patients, the initial differential diagnosis included metabolic, inflammatory, infectious, or neoplastic disease, with an eventual correct diagnosis obtained after catheter angiography had demonstrated arterialization of the deep venous structures, including the vein of Galen. All patients were successfully treated with endovascular embolization, with 1 patient requiring additional surgical treatment. We review the contemporary diagnostic evaluation and management of DAVF within the deep cerebral vasculature. With rapid diagnosis and treatment, a favorable outcome is possible.