Discrete glioneuronal malformative lesions in the foetal and infantile cerebral cortex.
Microdysgenesis is a term describing microscopic cortical cytoarchitectural abnormalities. Histologically this change shows an irregular glioneuronal tissue combination forming an abnormal structure of the cortex. The pathological features of this malformation are subtle and less well defined than other more distinctive cortical malformations. The clinical significance of these discrete glioneuronal malformations is controversial. Microscopic dysgenetic changes have been reported in cases with intractable epilepsy but similar changes may be seen in neurologically normal adults. The purpose of our study was the investigation of microdysgenetic lesions in the developing nervous system with regard to normal neuronal migration, differentiation and maturation. The post-mortem routine investigated foetal and infantile brains which were analysed histologically for the presence of discrete cortical malformations. A wide spectrum of cytoarchitectural glioneuronal malformations was found in the investigated material. We observed leptomeningeal glioneuronal heterotopias, subpial bands of heterotopic neurones, nests of ectopic neurones in the first cortical layer, neuronal and glial clusters, small foci with irregularity of laminar structure of the cortex. Microdysgenetic changes arose from an insult occurring in the later stages of cortical development and influencing the normal fate of neuroglial cells. Various types of focal morphological and cytoarchitectonial developmental abnormalities have been associated with behavioural and neuropsychological deficits in older infants.
Laure-Kamionowska
Milena
M
Department of Developmental Neuropathology, Medical Research Centre, Polish Academy of Sciences, Warszawa, Poland. milenak@cmdik.pan.pl
Maślińska
Danuta
D
Raczkowska
Barbara
B
D002540:Cerebral Cortex / Q000002:abnormalities* / Q000254:growth & development; D002828:Choristoma / Q000473:pathology*; D005333:Fetus; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008578:Meninges; D009457:Neuroglia / Q000473:pathology*; D009474:Neurons / Q000473:pathology*