Neuronal migration, cerebral cortical development, and cerebral cortical anomalies.
Cerebral cortical malformations are relatively common anomalies identified by neuroimaging and pathologically in patients with epilepsy and mental retardation. A disruption in neuronal migration during central nervous system development has been postulated as the pathogenesis for many of these disorders. Recently, the cell migration hypothesis has been proven accurate for lissencephaly, subcortical band heterotopia, and periventricular nodular heterotopia. Furthermore, advances in cellular and molecular biology have begun elucidating the fundamental mechanisms underlying these migration disorders. These data have resulted in redefining and recategorizing specific malformations based on their molecular genetic abnormality. In this review we shall discuss the current understanding of neuronal migration in the developing cerebral cortex, the evaluation of these patients, and attempt to describe the pathogenesis for several well-characterized human disorders of cell migration.
Pilz
Daniela
D
Institute for Medical Genetics, University Hospital of Wales, Cardiff.
Stoodley
Neil
N
Golden
Jeffrey A
JA
D043203:1-Alkyl-2-acetylglycerophosphocholine Esterase; D000818:Animals; D002465:Cell Movement; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000033:anatomy & histology / Q000196:embryology / Q000254:growth & development* / Q000502:physiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008869:Microtubule-Associated Proteins / Q000235:genetics / Q000378:metabolism; D008959:Models, Neurological; D009474:Neurons / Q000502:physiology*
D008869:Microtubule-Associated Proteins; D043203:1-Alkyl-2-acetylglycerophosphocholine Esterase; C084102:PAFAH1B1 protein, human