Venous encephalopathy

Abstract

Dural arteriovenous fistulae (DAVF) accounts for 10-15% of rare acquired intracranial vascular malformations which can present with a wide range of symptoms but DAVF presenting with onset of encephalopathy and cognitive decline as non-hemorrhagic neurological deficits has rarely been reported. A 44-year-old gentleman complained of headache followed by episodes of confusional behavior presenting to the emergency with two episodes of GTC seizures. He had history of cortical superficial venous sinus thrombosis and was treated with anticoagulants for one year. On admission his vitals were stable, GCS-6, no meningeal signs but decerebrate posturing with bilateral extensor planter response. CT venography reported non-opacification of superior sagittal, straight, left transverse and bilateral sigmoid sinuses (Fig1&2). Extensive collateral venous channels were seen in brain, parenchyma, basal cisterns and along the convexities. Few types of collateral were opacified in the arterial phase though intracranial arteries were normal. DSA revealed multiple DAVF (>5) fed by various arteries (Fig3&4). Endovascular embolization and recanalization was attempted but was not successful and patient was started on subcutaneous heparin. DAVF is referred to the abnormal communication between the intracranial artery and dural venous sinuses. DAVF can present with a myriad of clinical features so it is frequently misdiagnosed. The two unique features in this patient of DAVF were the presence of multiple sinuses and rare clinical presentation as venous encephalopathy. We assume that in this case the transformation in symptomatology from encephalopathy to seizures was heralded by increased arterial blood supply due to multiple sites of the fistulae.