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February 1969

Rod and Cone Responses in Sex-Linked Retinitis Pigmentosa

Author Affiliations

Bethesda, Md
From the Ophthalmology Branch and Perinatal Research Branch (Dr. Myrianthopoulos), National Institute of Neurological Diseases and Blindness, National Institutes of Health, Public Health Service, Dept of Health, Education, and Welfare, Bethesda, Md. Dr. Berson's present address is Department of Ophthalmology, Harvard Medical School, Massachusetts Eye and Ear Infirmary, and the Children's Hospital Medical Center, 300 Longwood Ave, Boston.

Arch Ophthalmol. 1969;81(2):215-225. doi:10.1001/archopht.1969.00990010217012

Psychophysical and electrophysiological testing are used to examine a family with sex-linked retinitis pigmentosa. Marked changes in the electroretinogram (ERG) precede any abnormalities visible with the ophthalmoscope. Both cone and rod responses are reduced in amplitude and delayed in implicit time. Cone responses are detectable after rod responses have disappeared. When rod function can be measured, scotopic luminosity curves and ERG responses to scotopically balanced lights indicate a normal rod action spectrum. The disease in males is widespread, whereas carrier females show loss of retinal function only in limited areas. Comparison of findings in carrier females with those in affected males provides support for the Lyon hypothesis.

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