頭蓋内異常血管網と高血圧のみられた幼児1剖検例

AN AUTOPSY CASE OF OCCLUSION OF THE CIRCLE OF WILLIS IN A CHILD

　いわゆる"特発性Willis動脈輪閉塞症"の小児剖検例は極めて少ない。本誌に昭和41年に有馬ら^1）が臨床報告した当時2歳1ヵ月の女児が6歳3ヵ月で死亡した。この症例の剖検所見について報告する。

Arima et al reported clinically a case of a two-year-old child developing spastic paraplegia, mental deterioration after several episodes of epileptic seizure. The patient was hypertensive with some proteinuria. Angiographically, carotid arteries could not be demostrated above syphone and in-creased typical abnormal vascular network at the base of the brain. (Arima et al.: Brain and Nerve, 18, 549, 1966)

The patient expired at the age of six year and three month.

The autopsy disclosed marked engorgement of tortous meningeal vessels over the entire brain (fig.2,3) and middle meningeal arteries were also dilated (fig. 5). Bilateral internal carotid arteries were narrow and stenosed with intimal fibrous thickening, heavy fatty deposits and isolated strands of newly formed elastic laminations. But there were no definite alterations in the media and adventitia (fig. 8). The distal portion of these arteries consisting of main portion of the circle of Willis showed myxomatous thickening with fat deposition and wavy elastic lamina suggestive of involution of arterial wall (fig 6, 9).

Fibrinoid necrosis of small arterial walls was seen in the granular layer as well as white matter of the cerebellum. Some associated with micro-glial proliferation. There were also scattered ball and ring hemorrhages (fig. 4, 7).