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Objective: To describe the diagnostic features of intracranial dural arteriovenous fistulae (DAVF) presenting with cervical cord or brainstem swelling. Methods: Retrospective case note and neuroimaging review of patients with angiographically confirmed DAVF diagnosed during January 2015–June 2020 at a tertiary neuroscience centre (Walton Centre NHS Foundation Trust, Liverpool, UK). Results: Six intracranial DAVF causing cervical cord or brainstem oedema (all males aged 60–69 years) and 27 spinal DAVF (88% thoracolumbar) were detected over a 5.5-year period. Significantly more patients with intracranial DAVF received steroids for presumed inflammatory myelitis than those with spinal DAVF (5/6 vs 1/27, p = 0.0001, Fisher's exact test). Several factors misled the treating clinicians: atypical rostral location of cord oedema (6/6); acute clinical deterioration (4/6); absence (3/6) or failure to recognise (3/6) subtle dilated perimedullary veins on MRI; intramedullary gadolinium enhancement (2/6); and elevated CSF protein (4/5). Acute deterioration followed steroid treatment in 4/5 patients. The following features may suggest DAVF rather than myelitis: older male patients (6/6), symptomatic progression over 4 or more weeks (6/6) and acellular CSF (5/5). Conclusion: Intracranial DAVF are uncommon but often misdiagnosed and treated as myelitis, which can cause life-threatening deterioration. Neurologists must recognise suggestive features and consider angiography, especially in older male patients. Dilated perimedullary veins are an important clue to underlying DAVF, but may be invisible or easily missed on routine MRI sequences. [ABSTRACT FROM AUTHOR]