February 1997

Vision in Patients With Leber Congenital Amaurosis-Reply

Author Affiliations

Boston, Mass

Arch Ophthalmol. 1997;115(2):294. doi:10.1001/archopht.1997.01100150295036

In reply  When we cited the article by Lambert et al1 in our recent article2 about Leber congenital amaurosis, we agreed with this conclusion: there are some patients with Leber congenital amaurosis who lose vision. However, as we noted,2 few patients who report deterioration of vision have, in fact, had it measured. Even in the seminal article by Lambert et al, counting fingers and hand movements are used as descriptors of vision. We all find these descriptors clinically useful, but they are not adequate for the quantitative assessment of the course of vision. As for the gratingletter acuity relationship in pediatric patients, it is well described.3We measured not only acuity, which is subject to a myriad of optical and neural effects, but also dark-adapted visual threshold, an index of peripheral retinal function. We demonstrated concurrent declines in acuity and dark-adapted visual sensitivity. It is unclear

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