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KMID : 0357919760100010103
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Korean Journal of Pathology
1976 Volume.10 No. 1 p.103 ~ p.112
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Sturge-Weber Syndrome
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지제근/Je. G. Chi
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Abstract
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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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