OBJECTIVES: Familial predisposition appears as an identified risk factor for @entity66 . The primary objective of our study was to assess @entity438 ( @entity438 ) recurrence rate in a population of familial @entity438 . Secondary objectives were first to analyse the inheritance categorisation/pattern of these families and second to assess the correlation between the aortic diameter on MRI and the aneurysmal characteristics. @entity1 AND METHODS: Over a period of 20 years (1990-2010), 26 @entity1 from 23 families, identified from a regional register, accepted to participate in this prospective trial in order to determine, the inheritance pattern, the screening of de novo @entity439 by CT angioscan, and the aortic mensuration by MRI. The transmission pattern was categorised into autosomal dominant inheritance, autosomal recessive and autosomal dominance with incomplete penetrance. The aortic diameter was measured: anatomic coverage in the caudo-cranial direction from the iliac arteries to the ventriculo-aortic junction. RESULTS: All 26 @entity1 [from 55.4  11.2 years, sex ratio female/male: 1.36] were reviewed after a mean follow-up of 7.9  6.6 years after the diagnosis of a @entity438 . The characteristics of this population were the diagnostic circumstances such as a @entity510 ( @entity510 ) in 14 (53.8%), the multiple locations in 10 (38.5%) and a giant @entity439 in 4 (15.4%). Four de novo @entity439 were diagnosed in 3 @entity1 (11.5%) after a mean follow-up of 22.3  4 years, which corresponds to an annual incidence of 1.9 (95% CI 1.4-2.6%). The transmission pattern was autosomal dominant in 16 (61.5%), recessive in 3 (11.5%) and not defined in 7 (26.9%). As regards the aortic diameter, a significant decrease in the aortic diameter was observed in @entity1 with an aneurysmal diameter superior to 10mm. CONCLUSION: The rate of de novo @entity439 justifies prolonged monitoring by imaging of these @entity1 with @entity438 . The narrowing of the terminal part of the aorta could be a hemodynamic factor involved into the @entity438 development.

XXXX , the relationship of the aortic diameter.

@entity1 :: ('9606', 'Species') :: ['patients', 'PATIENTS'] @entity66 :: ('MESH:D004194', 'Disease') :: ['cerebrovascular disease'] @entity510 :: ('MESH:D013345', 'Disease') :: ['SAH', 'subarachnoid hemorrhage'] @entity439 :: ('MESH:D000783', 'Disease') :: ['aneurysms', 'aneurysm'] @entity438 :: ('MESH:D002532', 'Disease') :: ['intracranial aneurysm', 'familial intracranial aneurysm', 'IA', 'cerebral aneurysm']
@entity438 :: (MESH:D002532,Disease) :: ['Familial intracranial aneurysm']
