Autosomal dominant retinitis pigmentosa: a log quotient analysis of the photopic and scotopic b-wave amplitude.
The relationship of the photopic and the scotopic b-wave amplitudes of the electroretinogram was studied in 85 normal subjects and 25 patients with autosomal dominant retinitis pigmentosa, in which one amplitude was at least 20 microvolts. The log quotient of their b-wave amplitudes--that is log of the photopic b-wave amplitude divided by the scotopic b-wave amplitude--was considered to represent the activity of cones relative to rods. The log quotient values had a normal gaussian distribution in the normal control eyes, while they formed two groups in the patients with autosomal dominant retinitis pigmentosa. In the first group (type 1), the scotopic b-wave was non-recordable while the photopic b-wave amplitude was larger than 20 microvolts in all cases, indicating that the log quotient is larger than 0.5 and that the rod system is much more severely affected than the cone system. The second group (type 2) had a log quotient smaller than 0.5 and its distribution almost overlapped the normal one, indicating more symmetrical damage in the cone and rod systems. The mean final rod threshold at 45 minutes for type 1 was significantly higher than that for type 2. The log quotient proved to be a useful index for analysing the cone and rod involvement and consequently provides a better understanding of autosomal dominant retinitis pigmentosa.
Iijima
H
H
Jules Stein Eye Institute, UCLA School of Medicine 90024-1771.
Martin
D A
DA
Heckenlively
J R
JR
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D004596:Electroretinography; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D008297:Male; D008875:Middle Aged; D010775:Photic Stimulation; D010786:Photoreceptor Cells / Q000503:physiopathology*; D012174:Retinitis Pigmentosa / Q000235:genetics / Q000503:physiopathology*