A Rare Case of Neurofibromatosis Type 1 Associated with Vertebral Arteriovenous Fistula and Moyamoya Syndrome

抄録

Objective: We report a rare case of neurofibromatosis type 1 (NF-1) presenting with myelopathy due to the vertebral arteriovenous fistula (AVF), and major intracranial artery occlusions with moyamoya-like vessels.

Case Presentation: A 66-year-old woman of NF-1 suffered from neck pain, tetraparesis, and sensory disturbance of gradual onset. Cervical MRI showed a huge flow void, and the spinal cord was strongly compressed by the dilated vessels. Vertebral angiography revealed the AVF at the level of C4/5 fed by the left vertebral artery and drained into the dilated epidural spinal vein between C2 and C5 via the intervertebral veins. In addition, carotid angiography showed the occlusion of the right internal carotid artery and the left anterior cerebral artery associated with moyamoya-like vessels. She underwent endovascular treatment with careful attention to the intraoperative hypotension and the AVF was completely occluded. Her neurological symptoms were cured after the treatment.

Conclusion: We experienced a rare case of NF-1 coexisted with the vertebral AVF and moyamoya syndrome. In such a complicated condition, discreet attention for possible cerebral hypoperfusion during the perioperative period should be paid for the successful treatment.