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Schizencephaly: A Neuronal Progression Disorder
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Why is it important?
Schizencephaly is a congenital cleft in cerebral mantle extending from pial surface to the lateral ventricle. It is lined by heterotopic grey matter. It is rare, with a prevalence of 1.48/1,00,000 births. In this case study, adult female brain specimen showed unilateral open lipped cleft - schizencephaly, in the left hemispheric perisylvian region communicating with the left lateral ventricular cavity. Cleft was lined by heterotopic grey matter. Schizencephaly is considered as the neuronal progression or migration disorder. It is also thought to be a brain segmentation disorder. This disruptive birth defect may have ischaemic - vascular injury at its root or it could be a presence of infection like Cytomegalo virus. Mutations in Homeobox gene EMX2 have been also implicated in this congenital anomaly. Our study indirectly confirms that unilateral cases of Schizencephaly may lead long life remaining undiagnosed till death.
Perspectives
Schizencephaly is a congenital cleft in cerebral mantle extending from pial surface to the lateral ventricle. It is lined by heterotopic grey matter. It is rare, with a prevalence of 1.48/1,00,000 births. In this case study, adult female brain specimen showed unilateral open lipped cleft - schizencephaly, in the left hemispheric perisylvian region communicating with the left lateral ventricular cavity. Cleft was lined by heterotopic grey matter. Schizencephaly is considered as the neuronal progression or migration disorder. It is also thought to be a brain segmentation disorder. This disruptive birth defect may have ischaemic - vascular injury at its root or it could be a presence of infection like Cytomegalo virus. Mutations in Homeobox gene EMX2 have been also implicated in this congenital anomaly. Our study indirectly confirms that unilateral cases of Schizencephaly may lead long life remaining undiagnosed till death.
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This page is a summary of: Schizencephaly: A Neuronal Progression Disorder, Indian Journal of Anatomy, January 2018, Red Flower Publication Private, Ltd.,
DOI: 10.21088/ija.2320.0022.7218.19.
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