BACKGROUND Tentorial dural arteriovenous fistulas (DAVFs) with perimedullary venous drainage causing cervical myelopathy are very uncommon conditions with an extremely aggressive behavior. When the characteristic radiological clues are missing, the unspecific clinical picture may cause delay and make the diagnosis challenging. OBSERVATIONS Here the authors report a case of a 58-year-old man who developed progressive spastic tetraparesis and dyspnea with an extensive mild enhancing cervical cord lesion initially oriented as a neurosyphilis-associated transverse myelitis. Acute worsening after steroid administration redirected the diagnosis, and a tentorial Cognard type V DAVF was elicited. The microsurgical disconnection process is described, and previously documented cases in the literature are reviewed. LESSONS If a DAVF is highly suspected, it is important to consider the possibility of its intracranial origin, and spinal as well as cerebral arteriography must be performed.

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