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Spontaneous epileptic manifestations in a DCX knockdown model of human double cortex.

Authors: Lapray, Damien  Popova, Irina Y  Kindler, Jennifer  Jorquera, Isabel  Becq, Hélène  Manent, Jean-Bernard  Luhmann, Heiko J  Represa, Alfonso 
Citation: Lapray D, etal., Cereb Cortex. 2010 Nov;20(11):2694-701. doi: 10.1093/cercor/bhq014. Epub 2010 Feb 17.
Pubmed: (View Article at PubMed) PMID:20164125
DOI: Full-text: DOI:10.1093/cercor/bhq014

Previous reports indicate that in utero knockdown of doublecortin (DCX) results in the genesis of a subcortical heterotopia reminiscent of the doublecortex observed in female patients with DCX mutations. It has also been shown that these rats display an increased susceptibility to convulsant agents and increased cortical neurons excitability; but it is presently unknown whether they display spontaneous seizures. Furthermore, the link between the size of heterotopia and the clinical manifestation remained to be elucidated. Using video-electrocorticogram recordings, we now report that DCX knockdown induces frequent spontaneous seizures commonly associated with myoclonic jerks in adult rats. Surprisingly, epilepsy occurred even in rats with very small subcortical heterotopias, as revealed by histological analysis of recorded animals. Moreover, the severity of the epileptic manifestations was positively correlated with both the size of the subcortical heterotopia and the age of recorded animals; thus, epileptic features were not detected in immature affected rats. In conclusion, our data demonstrate for the first time that subtle alterations can yield epilepsy and reveal a strong correlation between thicknesses of subcortical heterotopia, age of affected individuals and clinical impairment.

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CRRD Object Information
CRRD ID: 12904732
Created: 2017-05-19
Species: All species
Last Modified: 2017-05-19
Status: ACTIVE



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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.