Congenital hereditary optic atrophy is a rare but important specific entity, which is seldom recognized by most ophthalmologists. It is distinct from Leber's hereditary optic atrophy, particularly as to its age of onset, course, relative absence of associated physical abnormalities, and pattern of hereditary transmission. In the majority of the cases described in the literature there is moderate pallor of both optic discs with sufficient atrophy so that the cribiform plates are well made out. There is moderate to severe loss of vision. Physical abnormalities, other than optic atrophy, are not found consistently in affected persons. The optic atrophy is thought to be present at birth. Hereditary optic atrophy affects both sexes, and it may be transmitted in a dominant manner to successive generations through either the male or the female.
This type of congenital optic atrophy has been described in the literature by Gunn,1 Griscom,2 Thompson and
IVERSON HA. Hereditary Optic Atrophy. AMA Arch Ophthalmol. 1958;59(6):850–853. doi:10.1001/archopht.1958.00940070064006
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