Pathogenesis of Idiopathic Ventral Herniation of Spinal Cord: Neuropathologic Analysis

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.