Krings T, Geibprasert S, Luo C B, Bhattacharya J J, Alvarez H, Lasjaunias Pierre
Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin-Bicêtre, Paris, France.
Neuroimaging Clin N Am. 2007 May;17(2):245-58. doi: 10.1016/j.nic.2007.02.006.
The concept of segmental vascular syndromes with different, seemingly unrelated, diseases is based on the embryology of the neural crest and the mesoderm migration of cells that share the same metameric origin. Migrating patterns of these cells link the brain, the cranial bones, and the face on the same side. A somatic mutation developing in the region of the neural crest or the adjacent cephalic mesoderm before migration can, therefore, be postulated to produce arterial or venous metameric syndromes, including PHACES, CAMS, Cobb syndrome, and Sturge-Weber syndrome. Although these diseases may be rare, their relationships among each other and their postulated linkage with the development of the neural crest and the cephalic mesoderm may shed light on the complex pathology and etiology of various cerebral vascular disorders.
具有不同的、看似不相关疾病的节段性血管综合征的概念基于神经嵴的胚胎学以及具有相同体节起源的细胞的中胚层迁移。这些细胞的迁移模式将同侧的脑、颅骨和面部联系起来。因此，可以推测在迁移之前在神经嵴区域或相邻的头部中胚层区域发生的体细胞突变会产生动脉或静脉体节综合征，包括PHACES、CAMS、科布综合征和斯特奇-韦伯综合征。尽管这些疾病可能很罕见，但它们彼此之间的关系以及与神经嵴和头部中胚层发育的推测联系可能有助于揭示各种脑血管疾病的复杂病理和病因。
