Schizencephaly, consequence of a developmental vasculopathy? A clinicopathological report.
Schizencephaly was diagnosed by CT scan in a 3-month-old girl who died soon thereafter. The main neuropathological findings were the following: 1) A left frontal and a right parietal cleft were present which consisted in mesenchymal spaces taking the place of localized developmental defects of the cerebral mantel. 2) The gray matter lining the clefts was a microgyric cortical ribbon deeply infolded in the hemisphere without evidence of arrested neuronal migration. 3) However, true subependymal heterotopia were also recorded in areas distant from the microgyric ribbon. It is suggested that schizencephaly is initiated before the end of neuronal migration, at a time when the full thickness of the cerebral mantel can be easily destroyed by a localized malacic process, not later than 20 weeks of gestation. 4) Late ischemic insults to the cerebral cortex were observed, suggesting a long lasting process. Moreover, the death was due to an hematoma in the largest cleft, where atypical vessels were present. A developmental vasculopathy could be the cause for some cases of schizencephaly and could be life-threatening until the postnatal life.
Department of Pediatric Neurology, CHU Bicêtre, France.
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Landrieu
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Lacroix
D001921:Brain / Q000002:abnormalities* / Q000098:blood supply / Q000473:pathology; D002536:Cerebral Arteries / Q000473:pathology; D002540:Cerebral Cortex / Q000002:abnormalities / Q000098:blood supply / Q000473:pathology; D002543:Cerebral Hemorrhage / Q000473:pathology; D002550:Cerebral Veins / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007223:Infant; D002538:Intracranial Arteriovenous Malformations / Q000473:pathology*; D009474:Neurons / Q000473:pathology