Cajal-Retzius cells, inhibitory interneuronal populations and neuropeptide Y expression in focal cortical dysplasia and microdysgenesis.
Focal cortical dysplasia (FCD) and microdysgenesis (MD) are likely to represent abnormalities of radial neuronal migration during cortical development. We investigated the distribution of reelin-positive Cajal-Retzius cells, known to be important in the later stages of radial neuronal migration and cortical organization, in 12 surgical cases of both MD and FCD. Quantitation revealed significantly higher numbers of these cells in MD cases compared to controls. As the majority of cortical interneurones arise via tangential rather than radial migration, we studied the distribution and morphology of inhibitory interneuronal subsets immunolabelled for calbindin, parvalbumin and calretinin within these malformations. Frequent findings were a reduction of inhibitory interneurones in the region of FCD and abnormally localised hypertrophic or multipolar calbindin-positive interneurones in both FCD and MD. Neuropeptide Y immunostaining showed a striking increase in the density of the superficial plexus of fibres in both MD and FCD cases in addition to labelling of dysplastic neurones, which may represent an adaptive anti-convulsant mechanism to dampen down seizure propagation.
Department of Clinical and Experimental Epilepsy, Division of Neuropathology, Institute of Neurology, Queen Square, WC1N 3BG, London, UK. M.Thom@ion.ucl.ac.uk
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Harding
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Martinian
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Sisodiya
D000293:Adolescent; D000328:Adult; D064032:Calbindin 2; D064026:Calbindins; D015816:Cell Adhesion Molecules, Neuronal / Q000378:metabolism; D002465:Cell Movement / Q000502:physiology; D002540:Cerebral Cortex / Q000378:metabolism* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D016326:Extracellular Matrix Proteins / Q000378:metabolism; D005260:Female; D015870:Gene Expression; D006801:Humans; D007223:Infant; D007395:Interneurons / Q000378:metabolism* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins; D009421:Nervous System Malformations / Q000150:complications / Q000378:metabolism* / Q000473:pathology* / Q000601:surgery; D009433:Neural Inhibition / Q000502:physiology; D009434:Neural Pathways / Q000378:metabolism / Q000473:pathology; D009478:Neuropeptide Y / Q000096:biosynthesis*; D010320:Parvalbumins / Q000378:metabolism; D000091042:Reelin Protein; D064030:S100 Calcium Binding Protein G / Q000378:metabolism; D012697:Serine Endopeptidases
C576764:CALB2 protein, human; D064032:Calbindin 2; D064026:Calbindins; D015816:Cell Adhesion Molecules, Neuronal; D016326:Extracellular Matrix Proteins; D009419:Nerve Tissue Proteins; D009478:Neuropeptide Y; D010320:Parvalbumins; D000091042:Reelin Protein; D064030:S100 Calcium Binding Protein G; C000717127:RELN protein, human; D012697:Serine Endopeptidases