Development of the cortical dysplasia of type II lissencephaly.
A detailed neuropathological study of five immature brains with type II lissencephaly is reported. The cases described include two pairs of siblings. One infant survived for 2 months after birth, the others died at 18, 20, 20 and 32 weeks of gestation. This series of cases demonstrates the sequence in which the malformation develops from mid-gestation to post-natal life and shows that type II lissencephaly is not an intracortical malformation but is the result of massive glial and neuronal ectopia in the leptomeninges. This results from a failure of arrest of neuronal migration due to defects in the integrity of the pial/glial barrier.
Squier
M V
MV
Department of Neuropathology, Radcliffe Infirmary, Oxford.
D001921:Brain / Q000002:abnormalities* / Q000473:pathology; D001933:Brain Stem / Q000002:abnormalities / Q000473:pathology; D002525:Cerebellar Cortex / Q000002:abnormalities / Q000473:pathology; D002540:Cerebral Cortex / Q000002:abnormalities / Q000473:pathology; D005260:Female; D005333:Fetus / Q000473:pathology; D005865:Gestational Age; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011247:Pregnancy