Spinal Myxopapillary Ependymoma Presenting With Isolated Intracranial Hypertension: A Case Report

Myxopapillary ependymomas (MPEs) are rare, slow-growing spinal tumors that arise from the filum terminale and are classified as World Health Organization (WHO) Grade 2 tumors. They typically present with features of lower spinal cord or cauda equina compression, such as radiculopathy, limb weakness, or sphincter dysfunction. Presentation with features of increased intracranial pressure (IIP) is rare and has been reported in a few isolated cases. We present a case of a 21-year-old woman who presented with a two-week history of worsening headaches, neck stiffness, nausea, double vision, and tinnitus. Physical examination revealed bilateral abducens nerve palsies and papilledema, and lumbar puncture confirmed elevated cerebrospinal fluid (CSF) opening pressure. Brain imaging revealed features of IIP and showed superficial siderosis. In contrast, the spinal MRI scan revealed an intradural, extramedullary tumor at L1-L2 arising from the filum terminale, consistent with MPE. Surgical excision via L1-L3 laminectomy led to symptom resolution, and tumor histology confirmed a WHO Grade 2 MPE. The patient made a full recovery with no residual tumor on the three-month postoperative MRI. This case highlights a rare presentation of spinal MPE manifesting as IIP without typical lower limb neurology. The pathophysiology may involve elevated CSF protein and chronic subarachnoid haemorrhage, impairing CSF absorption. Spinal imaging should be considered in cases of unexplained intracranial hypertension, particularly when brain imaging is inconclusive.