ABSTRACT
The possibility that an atrophy of the optic nerve may be inherited became widely known to ophthalmologists in the classic report by Leber in 1871 (1). It is interesting that Leber considered it ‘‘congenitally induced’’ (1), although it usually appeared much later in life, and that it was considered a sex-linked disorder until very recently, when its nonmendelian transmission was documented. Leber’s disease remained the classic inherited optic atrophy. However, other inherited optic atrophies were described and do not seem to be uncommon. These diseases were placed in various different classifications, but it seems to me that the natural and logical classification should be based on the mode of transmission of the disease, with subdivision into the different entities. Table 1 provides such a basic classification of the inherited optic atrophies.
