Multiple dural arteriovenous fistulas presenting as rapidly progressive dementia

Dural arteriovenous fistulas (DAVFs) are important causes of neurological dysfunction and are many times misdiagnosed. Particularly in older populations, DAVFs may present with a selective cognitive dysfunction.

The authors describe a 70-year-old woman presenting with a rapidly progressive dementia, very similar in presentation to prion disease. Neuroimaging showed multifocal DAVFs associated with venous thrombosis and white matter changes, suggesting that impaired cerebral circulation due to venous hypertensive encephalopathy caused the patient's dementia. Prompt treatment of some of the abnormal shunts with endovascular embolization resulted in a clinically relevant improvement. Subsequent clinical improvement was achieved with anticoagulation, although no cause or predisposing factor was documented that could have led to the development of the venous thrombosis.

Neurologists should maintain a high degree of suspicion to avoid missing the diagnosis of DAVFs that are potentially treatable lesions.