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July 1968

Progressive Cone Degeneration, Dominantly Inherited

Author Affiliations

Bethesda, Md
From the Ophthalmology Branch, National Institute of Neurological Diseases and Blindness, National Institutes of Health, Bethesda, Md. As of July 1968 Dr. Berson's address will be Department of Ophthalmology, Children's Medical Center, 300 Longwood Ave, Boston 02115.

Arch Ophthalmol. 1968;80(1):77-83. doi:10.1001/archopht.1968.00980050079011

Psychophysical and electrophysiological techniques are used to evaluate a family with progressive cone degeneration. Affected patients have monophasic dark-adaptation curves with normal rod thresholds, absent color vision, and reduction or absence of the cone components with normal rod components in the electroretinogram (ERG). Scotopic luminosity curves and ERGs with scotopically balanced lights suggest that their rhodopsin has a normal absorption spectrum. The defect in the cone system does not appear to influence dark adaptation of the rod system. This report confirms the dominant genetic pattern of this disease.