[An unusual cause of acute myelopathy: a dural arteriovenous fistula at the craniocervical junction]. / Une cause exceptionnelle de myélopathie aiguë: la fistule artério-veineuse durale à la jonction cranio-rachidienne.

BACKGROUND: Dural arteriovenous fistulas (DAVF) account for 10% to 15% of all intracranial arteriovenous malformations. Since the first case published by Woimant et al. in 1982, many type V DAVF, i.e. with spinal venous drainage, have been reported. Fistulas located at the craniocervical junction (CCJ) however, are exceptional and only 10 cases of CCJ fistulas associated with myelopathy have been described. CASE REPORT: The authors present a 36-year-old male patient without previous medical history, suffering from acute myelopathy. Cervical MRI showed multiple serpiginous flow-voids along the cord surface and cerebral angiography disclosed a dural fistula of the CCJ fed by the right posterior meningeal and occipital arteries. The venous drainage was directed caudally towards the perimedullary veins. Embolization through the occipital artery, using cyanoacrylate, was performed and resulted in complete cure of the malformation with rapid clinical recovery. DISCUSSION: The authors discuss the pathophysiology and clinical consequences of intracranial DAVF with myelopathy (named V, m+), that are usually identical to those of spinal dural fistulas and related to intramedullary venous hypertension. Early treatment is essential to reverse the patient's myelopathy. Embolization, if technically possible, is the preferred treatment and cyanoacrylate remains the best embolic agent. Following glue deposition, systemic high-dose steroids should be administered to prevent edema. CONCLUSION: In conclusion, this is the first case of DAVF of the foramen magnum causing myelopathy to be detected early and cured by glue embolization alone, with rapid and total clinical recovery.