The postmortem findings in a two month old infant with the typical clinical features of Aicardi syndrome (i.e., infantile spasms, chorioretinal lacunae, and agenesis of the corpus callosum) are reported from the Division of Neurology, Saitama Children’s Medical Center, Saitama, Japan. The rostrum of the corpus callosum was absent and the roof of the dilated third ventricle was covered with a thin leptomeningeal membrane. Cortical heterotropias were found adjacent to the anterior horn of the right lateral ventricle and consisted of small immature neurons. The article includes a review of five autopsied patients with this syndrome previously reported in the literature. [1]

COMMENT. In these cases, a high incidence of EEG laterality and an asymmetry of pathological lesions are of interest. Three of six patients had focal agenesis of the corpus callosum and three had papilloma of the choroid plexus.