Wrobel C J, Oldfield E H, Di Chiro G, Tarlov E C, Baker R A, Doppman J L
Clinical Neurosurgery Section, National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, Maryland.
J Neurosurg. 1988 Dec;69(6):934-9. doi: 10.3171/jns.1988.69.6.0934.
Arteriovenous malformations (AVM's) of the spine commonly cause progressive myelopathy. Occasionally, myelography reveals serpentine filling defects characteristic of a spinal AVM, but an AVM or arteriovenous (AV) fistula cannot be demonstrated arteriographically, despite selective catheterization of all vessels known to have the potential of supplying the spinal cord and spinal dura. Often, and particularly in the setting of subacute or acute deterioration, this has been attributed to spontaneous thrombosis of the veins (the Foix-Alajouanine syndrome). Three patients are reported in whom intracranial dural AV fistulas, supplied by branches of the internal and external carotid arteries, drained into spinal veins and produced myelopathy. In one patient, motor and sensory deficits were limited to the lower extremities. In all three patients, disconnection of the fistula from its spinal venous drainage permitted arrest of a rapidly progressive myelopathy and partial recovery. These findings indicate that some patients who appear to have spinal cord AVM's but exhibit negative spinal arteriography are suffering from cranial dural AV fistulas and therefore need carotid as well as spinal arteriography. The considerable distance of these fistulas from the level of neurological expression supports venous hypertension as a pathophysiological mechanism of spinal cord injury. Interruption of a cranial dural fistula draining into spinal veins permits recovery of the myelopathy.
脊柱动静脉畸形（AVM）通常会导致进行性脊髓病。偶尔，脊髓造影可显示出脊柱AVM特有的蜿蜒状充盈缺损，但尽管对所有已知可能供应脊髓和硬脊膜的血管进行了选择性插管，仍无法通过动脉造影显示AVM或动静脉（AV）瘘。通常，特别是在亚急性或急性病情恶化的情况下，这被归因于静脉的自发性血栓形成（福-阿二氏综合征）。本文报告了3例患者，其颅内硬脑膜AV瘘由颈内动脉和颈外动脉的分支供血，引流至脊髓静脉并导致脊髓病。其中1例患者的运动和感觉障碍仅限于下肢。在所有3例患者中，将瘘与其脊髓静脉引流断开后，可使快速进展的脊髓病停止并部分恢复。这些发现表明，一些看似患有脊髓AVM但脊髓动脉造影呈阴性的患者实际上患有颅内硬脑膜AV瘘，因此需要进行颈动脉造影以及脊髓动脉造影。这些瘘与神经功能表现水平之间的距离较远，这支持静脉高压是脊髓损伤的病理生理机制。中断引流至脊髓静脉的颅内硬脑膜瘘可使脊髓病恢复。
