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 followi ng: 1) A left frontal and a right Parietal cleft were present which co nsisted in mesenchymal spaces taking the place of localized developmen tal defects of the cerebral mantel. 2) The gray matter lining the clef ts was a microgyric cortical ribbon deeply infolded in the hemisphere without evidence of arrested neuronal migration. 3) However, true sube pendymal heterotopia were also recorded in areas distant from the micr ogyric ribbon. It is suggested that schizencephaly is initiated before the end of neuronal migration, at a time when the full thickness of t he cerebral mantel can be easily destroyed by a localized malacic proc ess, 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, wher e atypical vessels were present. A developmental vasculopathy could be the cause for some cases of schizencephaly and could be life-threaten ing until the postnatal life.