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December 1989

Aicardi's Syndrome-Reply

Arch Ophthalmol. 1989;107(12):1724. doi:10.1001/archopht.1989.01070020806007

In Reply.  —We thank Dr van Dalen for his comments. He feels that, based on the chorioretinal, optic disc, and EEG findings, the patient we presented probably does not have Aicardi's syndrome. We maintain that the patient does have Aicardi's syndrome.The fundus photographs in the photo essay clearly show the typical punched-out chorioretinal lesions found in Aicardi's syndrome (compare these photographs with those in a report by Weleber et al1).Typical colobomas of the optic disc are limited to the inferior portion of the disc. However, the colobomas sometimes found in Aicardi's syndrome are atypical, involving the entire circumference of the disc,2 as seen in the photographs of our patient.Hypsarrhythmia on EEG is a variable feature of Aicardi's syndrome. In a review of 43 reported cases of the syndrome, Bertoni et al3 found that only 27 patients had hypsarrhythmia. Fariello et al reviewed 32 EEGs

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