The Importance of Retinal Electrophysiology in Ceroid Lipofuscinosis

Abstract

The two types of amaurotic idiocy, gangliosidosis and ceroid lipofuscinosis, are characterized by biochemical, ultrastructural, and electrophysiological differences. They are in fact two different clinical entities, both resulting from a congenital metabolic trouble.Six clinical cases of ceroid lipofuscinosis are presented and the diagnostic importance of the ERG is stressed.