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Ⅰ.はじめに
一卵性双生児に発症する破裂脳動脈瘤は稀であり,1942年O'Brienら10)の報告以来,現在までに12例の報告がなされているのみである.今回われわれは,脳動脈瘤家系の一卵性双生児に発症した破裂脳動脈瘤について症例報告をするとともに,過去の文献症例を含め,その臨床的特徴について検討した.また,近年,分子生物学的手法の発展ならびにヒトゲノムプロジェクトをはじめとする遺伝子データベースの整備により,多くの疾病について遺伝子解析がなされつつある.脳動脈瘤についても原因遺伝子の検討がなされているが,今回本症例についても若干の遺伝子的検索を試みてみた.
The hypothesis that genetic factors play a role in the genesis of cerebral aneurysms may be supported by clinical analysis of cerebral aneurysms in identical twins. In addition to reporting the present case,we review the literature on clinical features and genetical considerations in this matter.
A forty-two-year-old male presented with subarachnoid hemorrhage (SAH) due to a left MCA aneurysm. At the age of 39,his identical twin brother also had SAH due to a ruptured left MCA aneurysm. This identical twin brother and his father's sisters died of SAH. Venous blood of the patient and his parents was collected to determine apolipoprotein E,angiotensinogen,angiotensin-converting enzyme,and very low density lipoprotein receptor genes. This analysis suggested a strong genetic association of apolipoprotein E epsilon4 with the pathogenesis.
Including our cases,13 cases of cerebral aneurysms in identical twins have been reported. There was a total of 37 aneurysms. Six twins had the same sites of aneurysm. These aneurysms show a tendency to be small in size,and to cause a high frequency of SAH occurring at a similar age.
In young adults,cerebral aneurysms in identical twins tend to rupture. A screening diagnosis should be carried out as early as possible on an asymptomatic twin when the other has been discovered to have a ruptured cerebral aneurysm. Preventive treatment and systemic genome-wide linkage study should be conducted.
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