
Background: Dural arteriovenous fistulas (DAVFs) are rare, potentially curable causes of myelopathy. They are notoriously challenging to diagnose and carry a high rate of misdiagnosis, particularly when presenting with atypical features such as cervical cord involvement, younger age, acute symptoms, or unusual arterial feeders.
Case Presentation: A 64-year-old man presented with progressive neck pain, gait disturbance, and left-sided sensory symptoms. MRI showed extensive cervicomedullary and cervical cord edema with patchy intramedullary enhancement. CSF revealed elevated protein (170 mg/dL) with minimal pleocytosis. Demyelination was high in the differential, and corticosteroids were started. The patient worsened, developing right-sided weakness and urinary retention. Head and neck CTA was read as unrevealing; retrospective review identified subtle serpiginous vessels anterior to the cervicomedullary junction. DSA confirmed an atypical intracranial-spinal DAVF between a recurrent meningeal branch of the ophthalmic artery and the anterior and posterior spinal veins. Surgery led to clinical improvement.
Conclusion: This case highlights three generalizable lessons: (1) DAVFs, particularly atypical ones, can closely mimic inflammatory, demyelinating, or neoplastic cord lesions; (2) Persistent cord edema should raise suspicion for venous congestive myelopathy and prompt vascular imaging; (3) Low threshold for considering DAVF should be maintained, and when suspected, the entire neuraxis should be examined for atypical arterial feeders.