Janik Karolina, Manire Meredith A, Smith George M, Krynska Barbara
Shriners Hospitals Pediatric Research Center, Center for Neural Repair and Rehabilitation, Lewis Katz School of Medicine at Temple University, Philadelphia, PA, United States.
Department of Obstetrics and Gynecology, West Penn Hospital, Allegheny Health Network, Pittsburgh, PA, United States.
Front Cell Neurosci. 2020 Jun 30;14:201. doi: 10.3389/fncel.2020.00201. eCollection 2020.
Myelomeningocele (MMC) is the most common congenital defect of the central nervous system and results in devastating and lifelong disability. In MMC, the initial failure of neural tube closure early in gestation is followed by a progressive prenatal injury to the exposed spinal cord, which contributes to the deterioration of neurological function in fetuses. Prenatal strategies to control the spinal cord injury offer an appealing therapeutic approach to improve neurological function, although the definitive pathophysiological mechanisms of injury remain to be fully elucidated. A better understanding of these mechanisms at the cellular and molecular level is of paramount importance for the development of targeted prenatal MMC therapies to minimize or eliminate the effects of the injury and improve neurological function. In this review article, we discuss the pathological development of MMC with a focus on injury to the exposed spinal cord. We emphasize the need for a better understanding of the causative factors in MMC spinal cord injury, pathophysiological alterations associated with the injury, and cellular and molecular mechanisms by which these alterations are induced.
脊髓脊膜膨出（MMC）是中枢神经系统最常见的先天性缺陷，会导致严重的终身残疾。在脊髓脊膜膨出中，妊娠早期神经管闭合的最初失败之后是对暴露脊髓的渐进性产前损伤，这会导致胎儿神经功能恶化。尽管损伤的确切病理生理机制仍有待充分阐明，但控制脊髓损伤的产前策略提供了一种有吸引力的治疗方法来改善神经功能。在细胞和分子水平上更好地理解这些机制对于开发针对性的产前脊髓脊膜膨出治疗方法以最小化或消除损伤影响并改善神经功能至关重要。在这篇综述文章中，我们讨论脊髓脊膜膨出的病理发展，重点是暴露脊髓的损伤。我们强调需要更好地理解脊髓脊膜膨出脊髓损伤的致病因素、与损伤相关的病理生理改变以及诱导这些改变的细胞和分子机制。
