Electroretinographic findings in retinitis pigmentosa.
Full-field electroretinograms (ERGs) provide a basis for establishing the diagnosis of widespread progressive forms of retinitis pigmentosa in early life even at a time when fundus abnormalities visible with the ophthalmoscope are minimal or absent. Patients characteristically show delays in rod or cone b-wave implicit times or both, while patients with self-limited sector retinitis pigmentosa or stationary night blindness have normal b-wave implicit times. ERGs are presented to show their usefulness in detection of female carriers of X-chromosome-linked retinitis pigmentosa, in quantitating the natural course of all types of retinitis pigmentosa, and in detecting an abnormal rod ERG diurnal rhythm in the dominant form. Potential therapies for the retinitis pigmentosa associated with abetalipoproteinemia and Refsum's disease are also considered.
D000012:Abetalipoproteinemia / Q000503:physiopathology; D002940:Circadian Rhythm; D004596:Electroretinography; D005799:Genes, Dominant; D008040:Genetic Linkage; D006801:Humans; D010786:Photoreceptor Cells / Q000503:physiopathology; D012035:Refsum Disease / Q000503:physiopathology; D012174:Retinitis Pigmentosa / Q000235:genetics / Q000503:physiopathology*; D013997:Time Factors; D014960:X Chromosome