Interhemispheral Neuroepithelial (Glio-Ependymal) Cysts, Associated with Agenesis of the Corpus callosum and Neocortical Maldevelopment

Authors:
  • PBP.G. Barth
    P.G. Barth
  • HUH.B.M. Uylings
    H.B.M. Uylings
  • FSF.C. Stam
    F.C. Stam
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Abstract
Callosal agenesis and interhemispheral cysts containing ependyma, choroid plexus and glial cells are reported in a male infant. To the authors’ knowledge this represents the 4th recorded case. Other findings were: nodular neuronal heterotopias with fully differentiated spiny neocortical neurons, widespread neocortical microgyria, cerebellar hypoplasia and bony abnormalities of the foramen magnum and atlas. This study confirms that glioependymal cysts are heterotopias of embryonic ventricular epithelium. The existence of more than one type of heterotopia (i.e. neuronal and ependymal) in 1 individual is significant. This indicates an error of cell generation and cell migration affecting the original structure from which both cell types are derived: the embryonic (sub)ventricular zone.