Some Characteristics of the Electroretinogram in Hereditary Retinal Pigmentary Dystrophy. I. The Correlation of Recordability by Single Stimulus and Mode of Inheritance

Abstract

Single-stimulus electrorctinograms were recorded in dark adapted condition from patients with heriditary retinal pigmentary dystrophy of diffuse type. Responses were recordable from 27.6% of 123 patients. The recordability was significantly dependent upon the mode of inheritance: 59.4% of 32 patients with autosomal dominant inheritance showed recordable responses, whereas only 16.4% of 91 patients with autosomal recessive inheritance revealed detectable responses. An hypothesis seems valid that progression in autosomal dominant form is much slower than autosomal recessive form.

This work was supported by a research grant for retinitis pigmentosa from the Japanese Department of Health and Welfare.