[Oculocerebral anomalies in Walker's lissencephaly].
Two new cases of Walker's lissencephaly are reported. In this disease first described by Walker in 1942 important cerebral malformations and various ocular anomalies are associated. The main cerebral malformations consist of hydrocephalus and agyria, and the ocular anomalies concern the anterior segment as well as the retina which is frequently dysplastic. The originality of our cases is due to the retina which was not dysplastic but showed particular modifications which are discussed. Moreover, in the second case the cerebral cortex was rather microgyric. The aspect of the ocular lesions and of some of the cerebro-meningeal findings bring the authors to discuss the autosomal recessive inheritance proposed by some neuropathologists; these aspects can suggest a possible foetopathy.
D000015:Abnormalities, Multiple / Q000196:embryology / Q000235:genetics / Q000473:pathology*; D001921:Brain / Q000002:abnormalities* / Q000473:pathology; D002531:Cerebellum / Q000002:abnormalities; D002540:Cerebral Cortex / Q000002:abnormalities / Q000473:pathology; D002825:Chorioretinitis / Q000473:pathology; D005123:Eye / Q000473:pathology; D005124:Eye Abnormalities; D005260:Female; D005315:Fetal Diseases / Q000150:complications; D006801:Humans; D006849:Hydrocephalus / Q000473:pathology*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D012160:Retina / Q000473:pathology; D013577:Syndrome
Les anomalies oculo-cérébrales au cours de la lissencéphalie de Walker.