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Lissencephaly (agyria) on computed tomography.

K Ohno, T Enomoto, J Imamoto

    Journal of Computer Assisted Tomography
    |February 1, 1979
    PubMed
    Summary
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    This study details unusual brain imaging in infants with developmental delays and seizures. Findings suggest lissencephaly (agyria), a rare brain malformation caused by disrupted neuronal migration.

    Area of Science:

    • Neuroscience
    • Developmental Biology
    • Radiology

    Background:

    • Lissencephaly (agyria) is a rare congenital brain malformation characterized by a smooth brain surface.
    • It results from the interruption of neuronal migration during early fetal development.
    • Clinical presentations often include developmental delays, seizures, and abnormal motor posture.

    Observation:

    • Two infants presented with similar clinical symptoms: delayed milestones, seizures, decerebrated posture, and microcephaly.
    • Computed tomography (CT) revealed distinct neuroanatomical abnormalities.
    • Key CT findings included a small brain, widened subarachnoid space, smooth cerebral surface, uniformly enlarged ventricles, and wide sylvian cisterns.

    Findings:

    • The observed CT features are consistent with the pathological characteristics of lissencephaly (agyria).

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  • Specifically, the lack of insular opercularization was a notable observation.
  • These imaging findings correlate with the underlying disruption of neuronal migration.

    • Implications:

    • Early identification of lissencephaly through characteristic CT findings is crucial for timely diagnosis and management.
    • Understanding these imaging patterns aids in differentiating lissencephaly from other neurodevelopmental disorders.
    • Further research into the genetic and environmental factors influencing neuronal migration can inform future therapeutic strategies.