UNILATERAL SOMATIC AND INTRACRANIAL HYPOPLASIA

We report on clinical and MR findings in a woman with a peculiar disea se. She was studied from childhood to adulthood. She showed several an omalies affecting structures of ectodermal and mesodermal origin, Spec ific defects included right cerebral and cerebellar hypoplasia, right cerebral cortical polymicrogyria, agenesis of the corpus callosum, rig ht micro-ophthalmia and cataract, right breast hypoplasia, right upper and lower extremity hypoplasia, bilateral acral alterations affecting especially the middle phalanges, left hemifacial hypoplasia (probably secondary to the severe contralateral cerebral hemisphere lesion), me ntal retardation and partial epilepsy. MR disclosed right cerebral and cerebellar hypoplasia, right cerebral cortical polymicrogyria, agenes is of corpus callosum and right micro-ophthalmia. She did not exhibit either orbital cysts or cutaneous anormalities. The complex symptomato logy of unknown origin presented by this patient suggests a new develo pmental malformation consisting in hypoplasia of a complete hemibody.