Brandicourt P, Bonnet L, Béjot Y, Drouet C, Moulin T, Thines L
Service de neurochirurgie, CHRU de Besançon, université Bourgogne-Franche-Comté, 3, boulevard Alexandre-Fleming, 25030 Besançon, France.
Unité de neurologie vasculaire, CHRU de Besançon, université Bourgogne-Franche-Comté, 3, boulevard Alexandre-Fleming, 25030 Besançon, France.
Neurochirurgie. 2018 Mar;64(1):63-67. doi: 10.1016/j.neuchi.2017.11.004. Epub 2018 Feb 21.
Moya-Moya angiopathy is a neurovascular disease that predisposes to ischemic or hemorrhagic strokes. It is generated by a steno-occlusion of the terminal portion of the internal carotid arteries, which induces the development of abnormal neovessels in the deep regions of the brain. Some pathologies such as sickle cell disease, Down syndrome or Graves' disease may be associated with Moya-Moya angiopathy. These syndromic forms harbor several differences compared with idiopathic Moya-Moya disease.
We report the case of a young patient who presented with a syndromic form of Moya-Moya angiopathy after cranial radiation therapy for an optic glioma associated with type 1 neurofibromatosis treated by combined revascularization. We discuss the particularities of syndromic forms, in their presentation and management based on a review of the literature.
Many diseases can be associated with Moya-Moya syndrome. Symptomatic patients should undergo surgery, but the risk of postoperative complications appears to be greater than that encountered in patients with non-syndromic Moya-Moya angiopathy.
烟雾病是一种易引发缺血性或出血性中风的神经血管疾病。它由颈内动脉末端的狭窄闭塞引起，导致大脑深部区域出现异常新生血管。一些疾病，如镰状细胞病、唐氏综合征或格雷夫斯病，可能与烟雾病相关。这些综合征形式与特发性烟雾病存在一些差异。
我们报告了一例年轻患者，该患者在接受联合血运重建治疗与1型神经纤维瘤病相关的视神经胶质瘤后，出现了综合征形式的烟雾病。我们基于文献综述，讨论了综合征形式在表现和治疗方面的特殊性。
许多疾病可与烟雾综合征相关。有症状的患者应接受手术，但术后并发症的风险似乎高于非综合征性烟雾病患者。
