VON HIPPEL-LINDAU DISEASE

Von Hippel-Lindau disease is characterized by visual loss and manifestations related to cerebellar or spinal cord dysfunction. The basic lesion is angioma of retina, usually accompanied by hemangioblastoma of cerebellum, hemangioma of spinal cord, hypernephroma and cystadenomas of viscera may occur but are less frequent. Paradoxically, skin is not involved. This condition appears at adolescence or later.