Eibach Sebastian, Pang Dachling
Department of Clinical Medicine, Faculty of Medicine and Health Sciences, Macquarie University, Sydney, Australia.
Department of Neurosurgery, Macquarie University Hospital, Sydney, Australia.
J Korean Neurosurg Soc. 2020 May;63(3):327-337. doi: 10.3340/jkns.2020.0018. Epub 2020 Apr 27.
Junctional neurulation represents the most recent adjunct to the well-known sequential embryological processes of primary and secondary neurulation. While its exact molecular processes, occurring at the end of primary and the beginning of secondary neurulation, are still being actively investigated, its pathological counterpart -junctional neural tube defect (JNTD)- had been described in 2017 based on three patients whose well-formed secondary neural tube, the conus, is widely separated from its corresponding primary neural tube and functionally disconnected from corticospinal control from above. Several other cases conforming to this bizarre neural tube arrangement have since appeared in the literature, reinforcing the validity of this entity. The cardinal clinical, neuroimaging, and electrophysiological features of JNTD, and the hypothesis of its embryogenetic mechanism, form part of this review.
交界性神经胚形成是著名的原发性和继发性神经胚形成这一连续胚胎学过程的最新补充。虽然其确切的分子过程发生在原发性神经胚形成末期和继发性神经胚形成初期，仍在积极研究中，但其病理对应物——交界性神经管缺陷（JNTD）——已于2017年根据三名患者的情况进行了描述，这三名患者发育良好的继发性神经管（圆锥）与其相应的原发性神经管广泛分离，并且在功能上与上方的皮质脊髓控制脱节。此后，文献中出现了其他几例符合这种奇异神经管排列的病例，进一步证实了这一实体的存在。JNTD的主要临床、神经影像学和电生理特征及其胚胎发生机制的假说，构成了本综述的一部分。
