• Journal of Korean Neurosurgical Society
• /
• v.64 no.3
• /
• pp.374-379
• /
• 2021

Recent case reports of junctional neural tube defect (JNTD) which is a peculiar type of spinal anomaly showing the functional disconnection of the primary and secondary neural tubes has risen interest in the process of junctional neurulation (the connection between the two neural tubes) during development. This article summarizes the clinical features of the JNTD and reviews the literature on the basic research on junctional neurulation.

• Journal of Korean Neurosurgical Society
• /
• v.63 no.3
• /
• pp.327-337
• /
• 2020

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.

• Journal of Korean Neurosurgical Society
• /
• v.63 no.3
• /
• pp.272-278
• /
• 2020

Compared to any other decade, the last two decades have been the most dynamic period in terms of advances in the knowledge on spinal dysraphism. Among the several factors of rapid advancement, such as embryology during secondary neurulation and intraoperative neurophysiological monitoring, there is no doubt that Professor Dachling Pang stood high amidst the period. I review here the last two decades from my personal point of view on what has been achieved in the field of spinal dysraphism, focusing on occult tethered cord syndrome, lumbosacral lipomatous malformation, terminal myelocystocele, retained medullary cord, limited dorsal myeloschisis and junctional neural tube defect. There are still many issues to revise, add and extend. Profound knowledge of basic science is critical, as well as refined clinical analysis. I expect that young scholars who follow the footsteps of precedent giants will shed bright light on this topic in the future.

• Journal of Korean Neurosurgical Society
• /
• v.64 no.3
• /
• pp.386-405
• /
• 2021

Recent advancements in basic research on the process of secondary neurulation and increased clinical experience with caudal spinal anomalies with associated abnormalities in the surrounding and distal structures shed light on further understanding of the pathoembryogenesis of the lesions and led to the new classification of these dysraphic entities. We summarized the changing concepts of lesions developed from the disordered secondary neurulation shown during the last decade. In addition, we suggested our new pathoembryogenetic explanations for a few entities based on the literature and the data from our previous animal research. Disordered secondary neurulation at each phase of development may cause corresponding lesions, such as failed junction with the primary neural tube (junctional neural tube defect and segmental spinal dysgenesis), dysgenesis or duplication of the caudal cell mass associated with disturbed activity of caudal mesenchymal tissue (caudal agenesis and caudal duplication syndrome), failed ingression of the primitive streak to the caudal cell mass (myelomeningocele), focal limited dorsal neuro-cutaneous nondisjunction (limited dorsal myeloschisis and congenital dermal sinus), neuro-mesenchymal adhesion (lumbosacral lipomatous malformation), and regression failure spectrum of the medullary cord (thickened filum and filar cyst, low-lying conus, retained medullary cord, terminal myelocele and terminal myelocystocele). It seems that almost every anomalous entity of the primary neural tube may occur in the area of secondary neurulation. Furthermore, the close association with the activity of caudal mesenchymal tissue in secondary neurulation involves a wider range of surrounding structures than in primary neurulation. Although the majority of the data are from animals, not from humans and many theories are still conjectural, these changing concepts of normal and disordered secondary neurulation will provoke further advancements in our management strategies as well as in the pathoembryogenetic understanding of anomalous lesions in this area.