特发性脊髓腹侧疝的发病机制：神经病理学分析。
Pathogenesis of Idiopathic Ventral Herniation of Spinal Cord: Neuropathologic Analysis.
机构信息
Department of Neurosurgery, Radboud University Medical Center, Nijmegen, The Netherlands.
Department of Pathological Anatomy, Radboud University Medical Center, Nijmegen, The Netherlands.
出版信息
World Neurosurg. 2018 Jun;114:30-33. doi: 10.1016/j.wneu.2018.02.187. Epub 2018 Mar 10.
BACKGROUND
Idiopathic ventral herniation of the spinal cord is rarely seen as a cause of gradually increasing neurologic deficit. Its cause has never been clarified. It could be the result of a developmental disorder at 30- to 60-day gestational age. Neuropathologic analysis of herniated spinal cord tissue could probably support this hypothesis.
CASE DESCRIPTION
In a patient suffering from idiopathic ventral herniation of the spinal cord, a biopsy was performed in order to reduce the space-occupying effect. The biopsy was taken while intraoperative neuromonitoring was used. The patient recovered uneventfully without any additional deficit. Tissue analysis included histopathologic, immunohistochemical, and molecular examination (methylation profiling). The tissue did not appear as a normally functioning spinal cord; instead, a non-neoplastic glio-(neuronal) proliferation was found.
CONCLUSION
These findings support a developmental disorder as a cause for idiopathic ventral spinal cord herniation.
背景
特发性脊髓腹侧疝很少被视为逐渐加重神经功能缺损的原因。其病因从未被阐明。它可能是妊娠 30-60 天发育障碍的结果。疝出脊髓组织的神经病理学分析可能支持这一假说。
病例描述
在一例特发性脊髓腹侧疝患者中，为了减轻占位效应，进行了活检。在术中神经监测的同时进行了活检。患者恢复顺利，无任何额外的缺损。组织分析包括组织病理学、免疫组织化学和分子检查（甲基化分析）。组织没有表现出正常功能的脊髓；相反，发现了一种非肿瘤性的胶质（神经元）增生。
结论
这些发现支持发育障碍是特发性脊髓腹侧疝的原因。
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