脳動静脈奇形の家族内発生

Familial Occurrence of Intracranial Arteriovenous Malformation

I．はじめに

　脳動静脈奇形（以下AVM）の病因は，胎生期の血管形成異常に基づく先天性疾患と考えられている^2,4,7）．しかし．AVMの家族内発生は特殊な疾患の合併症として起こるものを除いては，世界で5家系の報告があるにすぎない^1,5,8-10）．最近われわれは，2家系4症例のAVMを経験したので，症例を報告するとともに，若干の文献的考察を加えた．

It is beyond that cerebral arteriovenous malfor-mation (AVM) is a congenital disease based on abnor-mal vascular formation in the embryonic stage.Nevertheless, familial occurrence of cerebral AVMshas been previously reported only in five familiesthroughout the world, when complications of specificdiseases such as Sturge-Weber-Dimitri disease, vonHippel-Lindaeu disease, and Osler-Rendu-Weberdisease were ruled out.

We have recently encountered four cases of twofamilial AVMs. In this paper, we review nineteencases of nine familial AVMs from literature includingour four cases from two families.