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September 1988

A Possible Explanation of the New Fundus Finding in Leber's Congenital Amaurosis

Arch Ophthalmol. 1988;106(9):1164-1165. doi:10.1001/archopht.1988.01060140324011

To the Editor.  —In the March 1987 issue of the Archives, Schroeder et al1 described two patients with poor vision and markedly reduced or extinguished photopic and scotopic electroretinograms. They also noted in these patients the presence of previously undescribed nummular retinal lesions. We wish to comment on their findings in light of a patient recently seen at our center.

Report of a Case.  —A 4½-year-old girl was referred to us with a complaint of decreased visual fields. The parents had noted that the child was unable to find objects placed in the periphery of her visual field since she was 1 year of age. The history disclosed that she had a moderate sensory neural hearing deficit that was discovered at the age of 3½ years. Previous physical and neurological examination findings were normal, and there was no evidence of mental retardation. A routine urinary amino acid screen done

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