Progressive diffuse arteriovenous malformation--case report

Abstract

A rare diffuse arteriovenous malformation (AVM) occurred in an 11-year-old boy with a 4-year history of transient ischemic attacks and weakness of the left extremities. Postcontrast computed tomography (CT) showed cord-like enhancement in the corpus callosum and pineal region. Magnetic resonance (MR) imaging demonstrated atrophy of the right hemisphere and multiple signal voids in the sulci. Cerebral angiography showed a diffuse AVM fed by the bilateral anterior cerebral and right middle cerebral arteries. The AVM nidus consisted of scattered nidi. Several draining veins appeared as multiple signal voids on MR images. No surgery was possible because of the scattered nidi. Left hemiparesis and mental retardation worsened, with progressive atrophy of the right hemisphere on serial MR images. Follow-up radiological examinations showed that the number of nidi increased, the draining vein enlarged, and the gyri calcified. The gyriform calcification on CT scans strongly resembled that seen in Sturge-Weber syndrome.