Figure - available from: Pediatric Radiology
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Lissencephaly type 1. a In this male fetus, MRI was performed at 32 weeks of gestation following identification of agyria on US. Axial T2-weighted MR image confirms agyria with very poor operculization of the insula. The pregnancy was terminated and a deletion of the LIS1 gene was identified. Image courtesy of Dr. Lucia Manganaro, Roma. b In a different patient, a 6-month-old boy (who did not have prenatal US), axial T2-weighted MR image shows the same finding. MRI was performed because of intractable seizures. A thin parieto-occipital T2-hyperintense layer, known as the cell-spare zone, is readily visible (arrows)
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The cerebral cortex represents a laminar structure of precisely spatially organized neurons in horizontal layers and vertical columns. Neurogenesis, neuronal migration and neuronal wiring are tightly regulated and coordinated procedures that result in the accurate formation of the human cerebral cortex. Abnormal fetal corticogenesis results in seve...
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