Sturge-Weber病のComputerized Tomography所見

COMPUTERIZED TOMOGRAPHY SCAN OF STURGE-WEBER DISEASE

I．はじめに

　Sturge-Weber病は，臨床上，血管性の顔面母斑（Port—wine Stain）の他に痙攣発作，片麻癖，知能低下および牛眼あるいは緑内症などの眼症状，同側性半盲等を呈する疾患であるが^1）7），Phakomatosisの1つとされ，その本態は先天性の顔面および頭蓋内のAngiomatosisである^3）7）。この頭蓋内の病変の存在ならびにその範囲を臨床上証明することは困難で，従来より主に頭蓋単純写上に現われる特有な石灰沈着や二次的変化から，その病変部位や拡がりを推定して来た。このほかに脳血管写上の変化^2）8）やBrain Scan^5）の所見についての検討がなされているが，正確な病変部位の把握には未だ不十分である。現在までのところ，本症のComputerized Tomo—graphy （以下CT） Scan上の所見についての詳細な報告はなく^6），今回我々は4例にCT Scanを施行，うち2例にはEnhancementも行い，知見を得たので報告する。

Four cases of Sturge-Weber disease underwentcomputerized tomography (CT) scan and in two ofthem, further enhancement study with infusion ofmeglumine diatrizoate in 65% at the dose of 2 ml/kg was performed. Their age was 5 m/o, 2 y/o, 8y/o and 20 y/o. The finding of CT scans weresummarized as follows.

1) CT scan was far useful than plain skullroentogenography to visualize the exact area ofcalcium deposits and its extent.

2) According to the extent of calcium depositson CT scan, Sturge-Weber disease was divided intotwo types, i. e., localized type in one case anddiffuse type in three cases.

3) Brain atrophy was more extensive than thearea of calcification particularly in diffuse type.

4) In spite of severe and diffuse involvement ofthe cerebral mantle, neither calcification nor atrophywas observed in the thalamus, the lenticular nucleusand the caudate nucleus. The architecture of suchdeep structures were well preserved.

5) In our series, no apparent ventricular dilatationwas found even in two cases of severe brain in-volvement in the form of cerebral hemiatrophy.

6) In two cases with infusion of contrast medium,the area of calcification were obviously enhancedand in one of them, significant enhancement of thechoroid plexus was noted on the affected side.

7) Contrary to our expectation, leptomeningealangiomatosis which is the sine qua non of thedisease and should cover the cerebral cortex wasnot visualized by contrast infusion method in thisseries.

These findings led us to conclude that CT scanwas an outstanding measure to detect secondarychanges of Sturge-Weber disease such as calcificationand brain atrophy and that this ivestigation wouldbe used in suspected cases.