Aicardi syndrome: MR assessment of brain structure and myelination.

Aicardi syndrome is characterized by the combination , in girls, of agenesis of the corpus callosum, epilepsy, and typical choroidal lacunae. Other associated brain abnormalities that have been described include gross asymmetry of the cerebral hemispheres, irregular ventricu lar contours caused by subependymal heterotopic nodules, cysts in the hemispheres and the posterior fossa, cerebellar hypoplasia, papillomas of the choroid plexus, holoprosencephaly, microphthalmia, and neuronal migrational abnormalities [1-5]. Detailed neuropathologic reports have not previously described abnormal myelination as a feature of this syndrome [1-3] . We report three cases in which MR Imaging provided detailed information on brain structure and myelination pattern .