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Generalized cortical dysplasia manifested by diffusely thick cerebral cortex.

G Marchal1, F Andermann, D Tampieri

  • 1Department of Neurology, McGill University, Montreal Neurological Hospital, Quebec, Canada.

Archives of Neurology
|April 1, 1989
PubMed
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Generalized cortical dysplasia, a mild lissencephaly form, causes intractable epilepsy and mental retardation. This congenital neuronal migration disorder is diagnosable with neuroimaging.

Area of Science:

  • Neuroscience
  • Neurology
  • Developmental Neuroscience

Background:

  • Rolandic macrogyria is linked to epilepsy and intellectual disability.
  • Focal cortical dysplasia is observed at the periphery of these lesions.
  • This study investigates the possibility of generalized cortical dysplasia.

Observation:

  • Two patients presented with intractable epilepsy and mental retardation.
  • They exhibited diffusely abnormal, thick cortex with shallow gyri and poor gray-white matter demarcation.
  • Biopsies revealed undifferentiated cortical layers 5 and 6 and poorly myelinated white matter with heterotopic neurons.

Findings:

  • Evidence suggests cortical dysplasia can be generalized, not just focal.
  • The observed syndrome represents a mild form of lissencephaly, a congenital neuronal migration disorder.

Related Experiment Videos

  • Surgical intervention (anterior callosotomy) in one patient led to significant epilepsy improvement.

    • Implications:

    • This generalized cortical dysplasia syndrome can be diagnosed pre-symptomatically using computed tomography (CT) or magnetic resonance (MR) scanning.
    • Understanding this condition aids in diagnosing and managing epilepsy and mental retardation.
    • It expands the spectrum of neuronal migration disorders and lissencephaly.