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October 1987

Diagnostic Criteria for Leber's Congenital Amaurosis-Reply

Author Affiliations


Arch Ophthalmol. 1987;105(10):1320-1321. doi:10.1001/archopht.1987.01060100021009

In Reply.  —In our article, we stated that "all patients presented with poor vision since birth or before 6 months of age." As we are sure Dr Foxman is aware, normal visual behavior is a qualitative assessment, at least during the first year of life. All patients were judged to have abnormal visual behavior for their age. These abnormalities ranged in chart notations from statements of "no visually directed behavior," "no response to visual stimuli," "will look toward light but does not follow well," "no fixation or following," to "fixation but no following." The essence of our statement and judgment, and the judgment of the examiner at the time, was that the children were not exhibiting normal visual behavior for their age. The electroretinograms obtained for all patients at the time that abnormal visual behavior was assessed were extinguished, with the exception of six, and we will discuss those.The

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