Blood-retinal barrier function in patients with cone or cone-rod dystrophy.
We assessed blood-retinal barrier function by vitreous fluorophotometry in 24 patients with either cone or cone-rod dystrophy who were segregated into three subgroups. Compared with a normal population, the patients demonstrated increased vitreous fluorescence (breakdown of the blood-retinal barrier) that positively correlated with peripheral pigmentary changes and an appreciable reduction in electroretinographic scotopic b-wave amplitude. A unique subgroup of three women with supernormal electroretinographic scotopic b-wave amplitudes to a high-intensity stimulus had an abnormal increase in permeability of the blood-retinal barrier even without peripheral pigmentary fundus changes.
D000293:Adolescent; D000328:Adult; D001790:Blood Physiological Phenomena; D002648:Child; D004596:Electroretinography; D005260:Female; D005451:Fluorescein Angiography; D005584:Fovea Centralis / Q000473:pathology; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D010539:Permeability; D010786:Photoreceptor Cells / Q000473:pathology*; D012160:Retina / Q000503:physiopathology*; D012162:Retinal Degeneration / Q000473:pathology / Q000503:physiopathology*