Neurological sequelae from brachiocephalic vein stenosis

Abstract

Stenosis of central veins (brachiocephalic vein [BCV] and superior vena cava) occurs in 30% of hemodialysis patients, rarely producing intracranial pathology. The authors present the first cases of BCV stenosis causing perimesencephalic subarachnoid hemorrhage and myoclonic epilepsy. In the first case, a 73-year-old man on hemodialysis presented with headache and blurry vision, and was admitted with presumed idiopathic intracranial hypertension after negative CT studies and confirmatory lumbar puncture. The patient mildly improved until hospital Day 3, when he experienced a seizure; emergency CT scans showed perimesencephalic subarachnoid hemorrhage. Cerebral angiography failed to find any vascular abnormality, but demonstrated venous congestion. A fistulogram found left BCV occlusion with jugular reflux. The occlusion could not be reopened percutaneously and required open fistula ligation. Postoperatively, symptoms resolved and the patient remained intact at 7-month follow-up. In the second case, a 67-year-old woman on hemodialysis presented with right arm weakness and myoclonic jerks. Admission MRI revealed subcortical edema and a possible dural arteriovenous fistula. Cerebral angiography showed venous engorgement, but no vascular malformation. A fistulogram found left BCV stenosis with jugular reflux, which was immediately reversed with angioplasty and stent placement. Postprocedure the patient was seizure free, and her strength improved. Seven months later the patient presented in myoclonic status epilepticus, and a fistulogram revealed stent occlusion. Angioplasty successfully reopened the stent and she returned to baseline; she was seizure free at 4-month follow-up. Central venous stenosis is common with hemodialysis, but rarely presents with neurological findings. Prompt recognition and endovascular intervention can restore normal venous drainage and resolve symptoms.