Aicardi's Syndrome; Radiologic Manifestations

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Abstract
Features of Aicardi''s syndrome include infantile spasms associated with pathognomonic chorioretinopathy, abnormalities of the corpus callosum with heterotopia of gray matter, and characteristic EEG findings. Vertebral abnormalities are also part of the syndrome, which is apparently limited to female infants. Subnormal mental development appears in all cases. Isolated cases in the literature were associated with facial asymmetry, plagiocephaly and the Dandy-Walker syndrome. Experience with 2 patients suggests that a search for cases among patients with chorioretinopathy and infantile spasms will show the syndrome to be more common than currently reported.

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