Intracranial dural arterio-venous fistula (DAVF) with spinal venous drainage is a rare clinical entity often underdiagnosed in the setting of unrevealing spinal angiography in a patient presenting with myelopathy. Here we present a case of a patient with acute myelopathy due to intracranial DAVF with spinal drainage who underwent successful Onyx embolization. 

The incidence of intracranial DAVFs is 10-15% of all intracranial vascular malformations.  DAVF classification is based on its venous drainage.  Clinical presentation with spinal venous drainage includes insidious onset of progressive myelopathy with paraesthesia, motor weakness, gait abnormality, and sphincter dysfunction. MRI typically reveals diffuse intramedullary T2 hyperintensity and prominent serpiginous intradural extramedullary flow voids. DSA of the intracranial and spinal vasculature is the gold standard imaging modality of choice. Treatment includes endovascular or surgical occlusion.

DAVF is a rare but treatable cause of an otherwise progressive myelopathy. DSA is warranted in patients with MRI findings consistent with spinal DAVF, particularly with an unrevealing spinal angiogram.