Bassou D, Darbi A, Atmane M, Jidal M, Elfenni J, Amezyane T, Benameur M, Elkharras A
Service d'imagerie médicale, hôpital militaire Mohammed-V, 10000 Rabat, Maroc.
J Neuroradiol. 2008 Dec;35(5):292-6. doi: 10.1016/j.neurad.2008.05.001. Epub 2008 Aug 9.
Type 1 neurofibromatosis is the most common of all the phakomatosis. It is a hereditary neurocutaneous syndrome that may involve any organ or system of the body. Central nervous system lesions are frequent and dominated by neoplasms and nonneoplastic hamartomatous lesions. Craniocerebral vascular abnormalities are relatively rare, most often occlusive or stenotic. The occurring of intracranial aneurysms during the neurofibromatosis type 1 gives rise to the question of the fortuitous aspect or not of this association, especially as the quasi-totality of the reported aneurysms in the literature are sacciform and most often unique. We report an original case of ectasic diffuse vasculopathy of the cerebral arteries associated with neurofibromatosis type 1 in a 43-year-old man presented with seizures.
1型神经纤维瘤病是所有错构瘤病中最常见的一种。它是一种遗传性神经皮肤综合征，可累及身体的任何器官或系统。中枢神经系统病变很常见，主要是肿瘤性和非肿瘤性错构瘤性病变。颅脑血管异常相对少见，最常见的是闭塞性或狭窄性病变。1型神经纤维瘤病期间颅内动脉瘤的发生引发了这种关联是否为偶然的问题，特别是因为文献中报道的动脉瘤几乎全是囊状的且大多为单发。我们报告了一例43岁男性患者的原始病例，该患者患有与1型神经纤维瘤病相关的脑动脉扩张性弥漫性血管病，并伴有癫痫发作。
