Retinal adaptation in infants and children with retinal degenerations

Abstract

Retinal adaptation of II infants and children with progressive retinal degenerations was studied. Diagnostic categories were Leber's congenital amaurosis (4 patients), infantile ceroid lipofuscinosis (1 patient), and retinitis pigmentosa syndromes (6 patients). Only limited appraisal of function was done electrophysiologically because all patients had extinguished or much attenuated electroretinal responses. Pupillography and psychophysical procedures did allow assessment of dark adapted sensitivity, and adaptation to steady background lights and after exposure to bleaching lights. From infancy onward, patients with these disorders had elevated dark adapted thresholds. Temporal integration appeared to be normal in patients with the RP syndromes and in a patient with Leber's congenital amaurosis. Steady background lights decreased retinal sensitivity, but less in patients than normal controls. The recovery from bleaches was abnormal in some patients. These preliminary results describe abnormal retinal adaptation in young patients with retinal degenerations and suggest underlying mechanisms that may be common to many retinal degenerative processes.