Retinitis pigmentosa can be followed over almost its entire course with narrow bandpassed computer averaged cone electroretinograms (ERGs). The long-term rate of decline of these responses can be described by an exponential function. A cone ERG actuarial table based on 1039 patients and 6553 visits is presented to show the estimated number of years for an average patient with a given 30-Hz cone ERG amplitude to decline to 0.05 μV (i.e. virtual blindness). The table is based on a projected rate of loss of 10% of remaining cone ERG amplitude per year for those not on treatment and 8.3% per year for those on treatment with vitamin A palmitate 15,000 IU/day. The table can be used to provide an estimate of the average long-term visual prognosis from a single visit; more precise estimates for a specific patient require several additional visits over 2-to 3-year intervals. Evidence is presented to support the idea that patients with a projected cone amplitude of 3.5 μV or greater at age 40 (about 25% of our patient population with typical retinitis pigmentosa) would be expected, on average, to retain some useful vision for their entire lives without treatment. Knowledge of the amount of remaining cone function in the ERG often reduces patient anxiety and helps patients plan for their future.
KeywordsRetinitis pigmentosa; Retinal degeneration; Retina; Cones; Electroretinogram; Vitamin A; Genetic disease; Night blindness It is an honor to receive the Ludwig von Sallmann Prize and a privilege to deliver this lecture in his memory. Dr. Ludwig von Sallmann was an internationally recognized leader in ophthalmic research. From my perspective one of his major contributions was the creation of a program on hereditary retinal diseases in the mid-1950s soon after he became Director of the Ophthalmology Branch of the National Institute of Neurological Diseases and Blindness. He and his colleagues established a clinic for patients with these diseases and developed a research program to understand the causes of these conditions and seek means for treatment. From 1966 to 1968, I examined patients directly under his supervision and studied the applications of electroretinographic testing in the early detection of these conditions, in particular retinitis pigmentosa. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. The Ganzfeld or full-field stimulus system for clinical ERG testing was first introduced in the Ophthalmology Branch of the National Institutes of Health. With this system a light source on top of a dome illuminates its inner surface through a diffuser thereby presenting a fair...