A rare case of intradural intramedullary cervical spinal neurenteric cyst in an adult: illustrative case.

Background: Neurenteric cysts are rare congenital lesions of the CNS, typically found in the cervical or thoracic spine and presenting in early life. Only 5% of spinal neurenteric cysts are intramedullary. The authors report the case of an intradural intramedullary neurenteric cyst in a 68-year-old woman.

Observations: The patient presented with 4 months of progressive left extremity numbness, weakness, and imbalance consistent with cervical myelopathy. MRI revealed a 1-cm cystic lesion in the intradural intramedullary cervical spine. Multilevel posterior cervical laminectomy for cyst resection was performed with gross-total resection. Histopathological analysis revealed squamous, columnar, and pseudostratified epithelium positive for epithelial membrane antigen and cytokeratin and negative for glial fibrillary acidic protein and S100 protein, consistent with a neurenteric cyst. Her symptoms and examination significantly improved after surgery.

Lessons: Neurenteric cysts comprise 1% of spinal lesions, and 5% are intradural intramedullary. Symptoms progress gradually but may fluctuate with cyst size changes. MRI is the preferred imaging, with histopathological analysis required for diagnosis. Operative intervention for gross-total resection is the recommended management. This case emphasizes that neurenteric cysts should be considered in older adults with cervical myelopathy and a cystic intramedullary mass and demonstrates the benefit of complete surgical removal. https://thejns.org/doi/10.3171/CASE25253.