Lejeune J P
Clinique Neurochirurgicale, Hôpital R.-Salengro, CHRU, Lille.
Neurochirurgie. 1997;43(5):292-8.
The pathogenesis of intracranial aneurysms is multifactorial, with acquired factors probably associated with genetic factors. Intracranial aneurysms may be associated with heritable connective tissue disorders, mainly polycystic kidney disease, Ehlers-Danlos syndrome, Marfan's syndrome, and pseudoxhantoma elasticum. In the recent literature, familial intracranial aneurysms have been documented by large epidemiological studies in Sweden and Finland, with a frequency as high as 6.7 to 10%. Familial intracranial aneurysms have special characteristics concerning the age of the patient at the time of rupture, the location of the aneurysm, the size of the aneurysm at the time of rupture. The frequency of familial intracranial aneurysms is an argument for systematic screening, especially for patients aged between 30 and 65, when a familial occurrence of the disease has been clearly established. Magnetic resonance angiography is the most convenient method of screening, before intra-arterial arteriography is performed when an aneurysm is suspected.
颅内动脉瘤的发病机制是多因素的,后天因素可能与遗传因素相关。颅内动脉瘤可能与遗传性结缔组织疾病有关,主要是多囊肾病、埃勒斯-当洛综合征、马凡综合征和弹性假黄瘤。在最近的文献中,瑞典和芬兰的大型流行病学研究记录了家族性颅内动脉瘤,其发生率高达6.7%至10%。家族性颅内动脉瘤在破裂时患者的年龄、动脉瘤的位置、破裂时动脉瘤的大小方面具有特殊特征。家族性颅内动脉瘤的发生率是进行系统筛查的一个理由,特别是对于30至65岁的患者,当该病的家族性发病已明确确定时。磁共振血管造影是最方便的筛查方法,在怀疑有动脉瘤时,先于动脉内血管造影进行。