To present a case report of reversible rapidly progressive dementia (RPD) due to a dural arteriovenous fistula (DAVF), and the outcome after treatment.

DAVFs are direct shunts between dural arteries and venous sinuses or cortical veins¹, and account for approximately 15% of all intracranial vascular malformations². The most usual presentation is pulsatile tinnitus, but approximately 11% of patients³ may present with non-hemorrhagic neurological deficits, such as RPD¹. Because diagnosis may be challenging⁴, proper treatment might be delayed, leading to serious cognitive sequelae⁵. Endovascular embolization (EE) remains the first-line treatment⁶, with excellent occlusion rates in most of cases⁷.

We describe a patient presenting with RPD due to a high grade DAVF, and his evolution after a two-staged EE.

A 43-year-old man’s family reported a 6-month history of headaches, hemiparesis, and cognitive decline with inappropriate behavior, inattention, word-finding problems, anterograde amnesia, and overall dysfunction leading to a severe impairment of his daily activities. COGNISTAT demonstrated errors in temporal orientation, sustained attention, following commands, verbal fluency, abstract thought, intention, planning, self-monitoring, and verbal and visual information retention. Computed tomography angiography revealed multiple parenchymal hyperdensities and enlarged venous sinuses. A diagnostic angiography confirmed the diagnosis of DAVF was confirmed, with feeders from the sphenoid branches from both middle meningeal arteries, draining into an enlarged vein of Galen.

EE was completed within two sessions via a combination of transarterial and transvenous approaches using Onyx and coils. Cognitive function improved rapidly within 9 days after the treatment and was discharged. A 60-days follow-up COGNISTAT demonstrated improvement in verbal fluency, executive functions, sustained attention, and orientation.

DAVFs are rare vascular lesions with many different presentations, including RPD; this is either due to cortical or thalamic involvement, with different cognitive profiles, depending on the venous congestion pattern. Prompt EE is necessary to prevent a continuous cognitive decline.