Fukawa O, Aihara H
Department of Neurosurgery, Iwaki Municipal Hospital, Fukushima, Japan.
No Shinkei Geka. 1987 Aug;15(8):911-9.
Twenty cases with 32 cerebral aneurysms in eight families of familial intracranial aneurysms are presented. The present 20 cases, 191 cases in 81 pedigrees in the prior 48 reports and our 692 cases of nonfamilial intracranial aneurysm are analyzed from the view points of sex incidence, age at onset, location of aneurysm, incidence of multiple aneurysms and familial relationship. In addition, the heredity of familial intracranial aneurysm is discussed. The characteristics of our 20 cases are summarized as follows: (1) Mean age of our cases was 53.8 years but it differed from that (41.7 years) of the prior cases. (2) However, five patients (25.0%) were diagnosed at younger age (under 39 years) as in the previous reports. (3) The incidence of multiple cerebral aneurysms was very high (40.0%). (4) Middle cerebral artery aneurysms showed a higher incidence (50.0%). Furthermore, we observed the familial incidence (0.7 - 3.2%) in the total intracranial aneurysms encountered at the same neurosurgical unit, the incidence (39.5%) of cerebral aneurysm among children in parent-children cases and that (44.4%) among siblings in sibling cases of familial intracranial aneurysm. Therefore, we suggested the possibility of incidental aneurysm in the remaining siblings in the cases of familial occurrence.
本文报告了8个家族性颅内动脉瘤家族中的20例患者,共32个脑动脉瘤。从性别发病率、发病年龄、动脉瘤位置、多发动脉瘤发病率及家族关系等方面,对目前的20例患者、此前48篇报道中81个家系的191例患者以及我们的692例非家族性颅内动脉瘤患者进行了分析。此外,还讨论了家族性颅内动脉瘤的遗传情况。我们20例患者的特点总结如下:(1)我们患者的平均年龄为53.8岁,但与此前患者的平均年龄(41.7岁)不同。(2)然而,与此前报道一样,有5例患者(25.0%)在较年轻的年龄(39岁以下)被诊断出来。(3)多发性脑动脉瘤的发病率非常高(40.0%)。(4)大脑中动脉瘤的发病率较高(50.0%)。此外,我们观察到在同一神经外科单位遇到的所有颅内动脉瘤中家族性发病率为(0.7 - 3.2%),在家族性颅内动脉瘤的亲子病例中儿童脑动脉瘤的发病率为(39.5%),在兄弟姐妹病例中兄弟姐妹间的发病率为(44.4%)。因此,我们提出在家族性发病病例中其余兄弟姐妹存在偶然动脉瘤的可能性。