title: Radial microcolumnar cortical architecture: maturational arrest or cortical dysplasia?
abstract: The fetal neocortical plate, from initiation of radial migration at 5 weeks' gestation until midgestation, exhibits radial microcolumnar architecture. Horizontal histologic layering or lamination becomes superimposed in the second half of gestation, although residua of the columnar pattern persist postnatally, particularly where the cortex bends: at the crowns of gyri and in the depths of sulci. Columnar architecture of the cortical plate in the first half of gestation mostly results from radial migration of neuroblasts, but the Cajal-Retzius neurons and GABAergic neuroblasts from tangential migration regulate a transition to horizontal lamination of the mature cortex. In children and adults, prominent columnar architecture is a feature of many focal cortical dysplasias and is now recognized as a distinctive pattern of focal cortical dysplasias in the new International League Against Epilepsy classification. It also occurs, however, in many genetic syndromes and chromosomopathic conditions, including 22q12 deletions (DiGeorge syndrome), in several primary cerebral malformations, in the contralateral cingulate gyrus in hemimegalencephaly, in cortical tubers of tuberous sclerosis, in the margins of porencephalic cysts resulting from prenatal infarcts, and in some inborn metabolic defects such as methylmalonic acidemia. Synaptophysin demonstrates both radial and horizontal lamination of synaptic layers. Persistent fetal cortical architecture is potentially epileptogenic. We conclude that columnar architecture is a maturational arrest in histogenesis of the neocortical plate and becomes a component of cortical dysplasia in the perinatal period. An initially physiological process thus becomes pathologic by virtue of advancing age, but traces of it persist in normal mature brains. It also occurs in many genetic and inborn metabolic diseases and after acquired ischemic insults of the fetal brain.
authors.affiliation: Department of Paediatrics (Neurology), University of Calgary Faculty of Medicine and Alberta Children's Hospital Research Institute, Calgary, Alberta, Canada. harvey.sarnat@albertahealthservices.ca
authors.forename: Harvey B
authors.initials: HB
authors.lastname: Sarnat
authors.forename: Laura
authors.initials: L
authors.lastname: Flores-Sarnat
mesh_terms: D000818:Animals; D059447:Cell Cycle Checkpoints / Q000502:physiology; D002454:Cell Differentiation / Q000502:physiology*; D002465:Cell Movement / Q000502:physiology*; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000254:growth & development / Q000473:pathology*; D006801:Humans; D054220:Malformations of Cortical Development / Q000473:pathology*