Non corneal closed eye electroretinography in healthy persons and in patients with neuronal ceroid lipofuscinosis (Stengel-Batten-Spielmeyer-Vogt disease).

Non corneal "closed eye" ERG was recorded with surface skin electrodes in 22 healthy subjects and in 7 patients with neuronal ceroid lipofuscinosis (Stengel-Batten-Spielmeyer-Vogt disease). Non corneal "closed eye" ERG was present in all normal subjects and the records comprised all the main subcomponents reported in the conventional corneal ERG. While the latencies of the a and b waves in the normal subjects differed but slightly from those reported from corneal ERGs, the amplitudes were reduced in the non corneal "closed eye" ERG. The parameters of the non corneal "closed eye" ERGs in the healthy subjects differed but slightly from those reported on non corneal "open eye" ERGs. When applied on patients with neuronal ceroid lipofuscinosis, the method proved clinically tenable and no sedation was required. The records show that ERG was absent in all but one of the patients tested.