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Ependymal abnormalities in lissencephaly/pachygyria

H B Sarnat1, H Z Darwish, P G Barth

  • 1Department of Pathology, University of Calgary Faculty of Medicine, Alberta, Canada.

Journal of Neuropathology and Experimental Neurology
|September 1, 1993
PubMed

Insights

Abnormal ependyma, the brain's inner lining, was found in children with neurodevelopmental disorders. These ependymal defects may play a key role in conditions like lissencephaly and Walker-Warburg syndrome.

Area of Science:

  • Neuroscience
  • Developmental Biology
  • Pathology

Background:

  • The ependyma forms the brain's ventricular lining and is crucial for neurodevelopment.
  • Disorders of neuroblast migration lead to severe brain malformations.
  • Previous research has not extensively detailed ependymal pathology in these conditions.

Purpose of the Study:

  • To investigate ependymal morphology and immunohistochemistry in children with neuroblast migratory disorders.
  • To identify common ependymal abnormalities across diverse conditions including lissencephaly and Walker-Warburg syndrome.
  • To explore the potential role of ependymal dysfunction in the pathogenesis of these disorders.

Main Methods:

  • Examination of ependymal tissue from eight children with various neuroblast migratory disorders.
  • Morphological analysis of ependymal structure, including heterotopic ependymal rosettes and subependymal nodules.
  • Immunohistochemical staining for proteins such as glial fibrillary acidic protein, S-100 protein, cytokeratin CK-904, and vimentin.

Main Results:

  • Strikingly similar morphological and immunohistochemical abnormalities of the ependyma were observed in all cases.
  • Ependymal discontinuities were disproportionate to ventriculomegaly.
  • Persistent expression of S-100 protein, cytokeratin CK-904, and vimentin was noted in ependymal cells, indicating abnormal maturation.

Conclusions:

  • Abnormal ependymal development is a consistent finding in diverse neuroblast migratory disorders.
  • Ependymal abnormalities may contribute to disturbances in neuronogenesis, axonal guidance, and neuroblast migration.
  • The ependyma could be a primary factor in the pathogenesis of lissencephaly and related conditions.

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