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The Korean Journal of Pathology 1976;10(1): 103-112.
스터지-웨버 증후군
Sturge-Weber Syndrome
Based on two autopsy and six lobectomy materials gross and microscopic findings of Sturge-weber syndrome(Encephalofacial angiomatosis) are presented and discussed along with the descriptions already available in the literature. Focal glial scar often seen in the outer layers of the involved cortex appears to be progressive together with neuronal loss. These changes are seen even in the younger age group (9 months and 1 9/12 years). Involvement of the skull bone over the leptomeningeal angioma was present in one case, and there were two cases that had angiomatous involvement of the choroid plexi of the lateral ventricles of the affected sides. These findings were interpreted to support the view that the basic malformation in the encephalotrigeminal angiomatosis might be related to a persistence of the primordial vascular plexus, described by Streeter in the human embryo.
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