Sturge-Weber氏病の1例

Sturge-Weber's Syndrolne:Report of a Case

　Sturge-Weber氏病は顔面に於ける血管腫或は母斑，緑内障或は牛眼，脳に於ける特異な石次化像を主徴とし，癲癇様痙攣発作，智能低下等を随伴症状とする一連の症候群に対する名称である。

　たまたま頭部外傷によつて入院した患者に，右顔面血管腫と共に頭部レ線像により右後頭部の石灰化像を発見し，Sturge-Weber氏病である事を確め得たので報告する。

A 49-years-old farmer. On admission to our clinic on account of the accidental head trauma, the diagnosis of Sturge-Weber's syn-drome was established owing to his chara-cteristic facial angiomatosis associated with intracerebral calcification.

No evidences of hereditary and familial in-cidence were found. The facial angiomatosis was found at birth in the right frontal area, but no epileptic seizure and no mental retar-dation were recorded up to the present. On roentgenographic examination the intracere-bral calcification of the right occipital region and the enlargement of the diploic vein were revealed.

Pneumoencephalographic examination re-vealed the degenerative changes in the cere-bral parenchyma.

Electroencephalographically some differen-ces were observed between the right and left temporal lead in hyperventilation and pentazol activation.

Eosinopenic response to adrenalin and AC TH was normal, and plasma level of 17 OHCS, 17 KS excretion in urine, and PHI were in the normal range.

Strabism of the right eye was also noted.