Magnetic resonance imaging of the brain in Aicardi's syndrome. Report of 20 patients.

Aicardi's syndrome consists of multiple anomalies of the eye, brain, and axial skeleton in females, and is associated with early-onset infantile spasms and severe developmental delay. For the present study, magnetic resonance images of 20 patients with Aicardi's syndrome were obtained and the neuroimaging findings catalogued. These were compared to previously reported results and the findings correlated with current theories of fetal brain development. The results revealed a high frequency of cortical migration defects (94%) and a wide variability in the callosal defect, migrational abnormalities, and other findings in Aicardi's syndrome.