多発性のう胞腎と合併する脳動脈瘤

THE ASSOCIATION OF POLYCYSTIC KIDNEYS WITH INTRACRANIAL ANEURYSMS

I．はじめに

　家族性に発生する多発性腎のう胞は，しばしば肝のう胞，膵のう胞，さらにウイリス動脈輪の動脈瘤を合併することが知られている。一方脳動脈瘤の先天性発生説の一根拠として，多発性のう胞腎に脳動脈瘤が合併する頻度が高いことがあげられている。多発性のう胞腎と脳動脈瘤との合併例は，1901年Boreliusの報告^3）に始まり，諸外国では数多くの報告がみられているが，わが国においては，渉猟した限りでは，報告例がなく，免疫学的検索もまつたくされていない。最近，多発性のう胞腎の一家系の一人が前交通動脈瘤の破裂によつて死亡した症例を経験したので，のう胞腎と脳動脈瘤の関係について検討するとともに，1975〜1978年の日本病理剖検輯報^15〜18）の剖検95,854例（検索中と記載のないものを除く）から，多発性のう胞腎と脳動脈瘤との合併例について抽出して，検討を試みたので報告する。

A 48 year-old man is presented who died of rupture of intracranial aneurysm. Autopsy findings revealed a ruptured anterior communicating artery aneurysm associated with polycystic kidneys and polycystic liver.

Epidermiological review is performed using "Annual of the Pathological Autopsy Case in Japan"(Vol. 17-20). In 92854 autopsy cases, there are 243 cases of polycystic kidney, 1542 cases of intracranial aneurysm and 10 cases of association with both of them.

Several points come clear from comparing our data with foreign ones, as follows;

1) The frequency of polycystic kidney (0.25%) is almost the same between Japan and foreign country.

2) The frequency of intracranial aneurysm (1.6%) is higher in Japan.

3) The frequency of association with both (0.01%) is lower in Japan.

4) The frequency of intracranial aneurysm appears to be higher in cases suffered from poly-cystic kidney than in others.

We suspected that cerebral aneurysm formation is caused by hypertension due to polycystic kidney, although in many reports, the association of poly-cystic kidney and cerebral aneurysm is explained to be maldevelopment.

The operation of cerebral aneurysm with poly-cystic kidney is the same as that without polycystic kidney. But the control of hypertension and renal function is necessary during and after operation.