Diagnostic Criteria for Leber's Congenital Amaurosis

Abstract

To the Editor. —The number of articles on Leber's congenital amaurosis has increased in recent years. In the March 1987 issue of the Archives, Schroeder and colleagues presented another 43 patients with this diagnosis. They noted that mental retardation was a commonly associated handicap, in contrast with the findings of Nickel and Hoyt.¹ This is an extremely important issue, because the parents of young children with this disorder are not only told of the unfavorable ocular diagnosis, but they may also be told that there is a high chance of mental retardation. However, like others, Schroeder et al also concluded that Leber's congenital amaurosis is not a single entity but a constellation of eye findings that are associated with several diseases. The diagnosis of mental retardation in young, blind children is exceptionally difficult. Unfortunately, and possibly because of space limitations, Schroeder at al did not provide us with detailed