Ventral porencephaly: A cerebral defect associated with multiple congenital anomalies |
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Authors: | R Malcolm Stewart Roger S Williams Petr Lukl Jean Schoenen |
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Institution: | (1) Departments of Neurology and Neuropathology, Massachusetts General Hospital, 200 Trapelo Road, 02154 Waltham, MA, USA;(2) the Eunice Kennedy Shriver Center for Mental Retardation, Inc., Boston, MA, USA;(3) Department of Pathology, Cambridge Hospital, Harvard Medical School, Boston, MA, USA |
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Abstract: | Summary An infant with multiple congenital anomalies was found at autopsy to have a porencencephalic defect on the ventral surface of the left frontal lobe. The intracranial defect was seen in association with an anomalous configuration of the circle of Willis. The zone of tissue destruction corresponded to the vascular territory of the anterior choroidal and lenticulo-striate branches of the proximal middle cerebral arteries, which were absent on the left. The developmental anomaly of the circle of Willis may have predisposed to tissue destruction by compromising cerebral perfusion at midgestation, a stage of rapid brain growth.Supported in part by USPS grants. M 16674, MH 25515 and MH 01215 |
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Keywords: | Porencephaly Perfusion failure Circle Willis |
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