NON CORNEAL CLOSED EYE ELECTRORETINOGRAPHY IN HEALTHY PERSONS AND IN PATIENTS WITH NEURONAL CEROID LIPOFUSCINOSIS (STENGEL - BATTEN - SPIELMEYER - VOGT DISEASE)

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Abstract
Noncorneal closed eye ERG [electroretinogram] was recorded with surface skin electrodes in 22 healthy subjects and in 7 patients with neuronal ceroid lipofuscinosis (Stengel-Batten-Spielmeyer-Vogt disease). Noncorneal 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 ERG, the amplitudes were reduced in the noncorneal closed eye ERG. The parameters of the noncorneal closed eye ERG in the healthy subjects differed slightly from those reported on noncorneal open eye ERG. When applied on patients with neuronal ceroid lipofuscinosis, the method proved clinically tenable and no sedation was required. ERG was absent in all but 1 of the patients tested.