Posterior reversible encephalopathy syndrome: A rare neurological manifestation in Von Hippel-Lindau disease

Abstract

We report on a 34-year-old woman, who was recently diagnosed with Von Hippel-Lindau disease (VHL), genetically confirmed. At this moment, she presented with an acute history of arterial hypertension, headache, cortical blindness and epilepsy. On the basis of clinical and magnetic resonance imaging (MRI) criteria the diagnosis of a posterior reversible encephalopathy syndrome (PRES) was made. A iodine 123-labeled metaiodobenzylguanidine (MIBG) scan revealed the presence of bilateral adrenal pheochromocytomas.