|
KMID : 0377619700190030193
|
|
Korean Jungang Medical Journal
1970 Volume.19 No. 3 p.193 ~ p.195
|
|
|
Report of a Case of Sturge-Weber’s Syndrome
|
|
Hahn, Yoon Sun
Kim, Won Bae/Kim, Hwa Don
|
|
|
Abstract
|
|
|
Sturge-Weber´s syndrome is a ectodermal dysplasia with two essential features of cavernous type of cutaneous angioma of the face and focal venous angiomatosis of the pia-arachnoid over the cerebral cortex.
The vessels consist of a layer of endothelium backed up by some collagenous tissue.
Calcification is not present in these thin walled abnormal vessels, although there may be occasional small plaques or calcification in the more normal small arteries feeding the area.
The cerebral cortex underlying the ventous angioma tosis is characterized by shrinkage of the gyri, an increased number of blood vessels with excessive perivascular fibrosis and widespread deposition of calcium.
It is assumed that the shrinkage of the cerebral cortex in the involved areas is not due to congenital deficiency of cerebral substance in it-self, but is due to progressive gliosis and calcification.
We are reporting one classic case of Sturge-Weber´s syndrome with cutaneous nevus, convulsive seizure mental retardation and neurological findings. X-rays of skull revealed the characteristic "Railroad Track" appearance.
|
|
|
KEYWORD
|
|
|
|
|
|
FullTexts / Linksout information
|
|
|
|
|
|
Listed journal information
|
|
|
|