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Related Experiment Videos

Brain functional imaging SPECT in agyria-pachygyria

C Chiron1, R Nabbout, F Pinton

  • 1Neuropediatric Department, INSERM U29, Hospital Saint-Vincent de Paul, Paris, France.

Epilepsy Research
|June 1, 1996
PubMed
Summary

Agyria-pachygyria, a brain malformation, shows abnormal frontal lobe blood flow in children, suggesting impaired postnatal brain development and persistent infantile spasms (IS). This differs from other causes where IS typically resolve earlier.

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Area of Science:

  • Neuroscience
  • Developmental Biology
  • Pediatric Neurology

Background:

  • Agyria-pachygyria (lissencephaly type I) is a diffuse cortical malformation associated with refractory infantile spasms (IS).
  • Unlike other causes, IS in lissencephaly often persist beyond the first decade.
  • Understanding postnatal brain development in lissencephaly is crucial for explaining persistent epilepsy.

Purpose of the Study:

  • To investigate the functional postnatal development of the lissencephalic cortex.
  • To compare regional cerebral blood flow (rCBF) in children with lissencephaly to controls and children with cryptogenic IS.
  • To explore the relationship between rCBF patterns and the persistence of infantile spasms.

Main Methods:

  • Regional cerebral blood flow (rCBF) was measured using Single Photon Emission Computed Tomography (SPECT) and 133Xe.

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  • 14 children with lissencephaly (4 months–12 years) were studied.
  • Comparisons were made with age-matched normal children and children with cryptogenic IS (3 months–3 years).

    • Main Results:

    • Lissencephalic patients exhibited a higher frontal (FR)/parieto-temporo-occipital (PTO) rCBF ratio compared to controls and cryptogenic IS patients (P < 0.001).
    • This elevation was primarily due to increased frontal rCBF, especially in younger patients (< 3 years).
    • In contrast to controls, FR/PTO and FR rCBF remained stable with age and topography in lissencephalic patients, indicating a lack of normal developmental changes.

    Conclusions:

    • The findings suggest a deficit in normal postnatal brain development in agyria-pachygyria.
    • Altered frontal lobe development and blood flow may contribute to the characteristic persistent epileptic spasms in this condition.
    • This study highlights a potential mechanism for the intractable epilepsy seen in lissencephaly type I.