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Radial neuronal assemblies, ectopia and necrosis of developing cortex: a case analysis.

Abstract

Reduced size of convolutions and midcortical laminar necrosis are approximately co-extensive in the cerebral hemispheres of a child, one of twins, dying at 18 months. Because the underlying laminar arrangement of neurons and the basic gyral pattern are normal, the cortical damage probably occurred not earlier than the third trimester of gestation. Neurons surviving above and below the zone of tissue necrosis, like their homologs in normal cortex, are entrained in multineuronal radial assemblies. Below the zone of necrosis the relative positions of radially adjacent neurons are unaltered. Above, however, in places where the molecular layer is reduced in width, neurons are displaced radially outward toward the pial surface. In places the pia is breached and bridged by a mesenchymal-glial cicatrix. Where this has happened neurons have migrated beyond the cerebral boundary and have established an ectopia in the subarachnoid compartment. These observations suggest that relatively undifferentiated intracortical neurons are held in radial assemblies by bonds which prevent their tangential displacment. The molecular layer appears to serve as a barrier to their radial displacement.

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BibTeXRIS

V S Caviness, P Evrard, G Lyon. 1978-01-19. Radial neuronal assemblies, ectopia and necrosis of developing cortex: a case analysis.. https://doi.org/10.1007/bf00689559

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Prenatal diagnosis of malformations of cortical development by dedicated neurosonography.

OBJECTIVE: Malformations of cortical development (MCD) are rarely diagnosed in utero. We describe and compare the ultrasonographic and pathology findings in a cohort of fetuses with MCD. METHODS: Fetuses with MCD were identified among all fetuses evaluated for suspected brain anomalies at the Fetal Neurology Clinic, and the ultrasonographic findings were compared with the results of the pathology examination. RESULTS: We suspected the presence of MCD by ultrasonography in 23 fetuses. The mean gestational age at the time of ultrasound diagnosis was 26.2 (range, 18-40) weeks. The ultrasonographic findings leading to the diagnosis of MCD were abnormally overdeveloped gyri and sulci for gestational age (n = 7), delay in sulcation (n = 5), abnormally thin cortex (n = 5) abnormally wide and broad sulci (n = 3), bulging into the lateral ventricle (n = 1), cortical cleft (n = 1), and multiple intraparenchymal echogenic nodules (n = 1). All fetuses had associated central nervous system (CNS) and/or non-CNS anomalies. Pathology examination (performed in 17 fetuses) confirmed MCD in 16. CONCLUSIONS: Cortical malformations can be diagnosed in utero by ultrasonography based on the presence of specific deviations from the normal pattern of development. The identified cases may represent the more severe forms in the MCD spectrum. The pathology findings do not always conform to the current classification systems of MCD but help in differentiating between possible genetic and acquired etiologies and in some cases provide a definitive syndromic diagnosis.

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