Jellema K, Tijssen C C, van Gijn J
Department of Neurology, St Elisabeth Hospital, Tilburg, The Netherlands.
Brain. 2006 Dec;129(Pt 12):3150-64. doi: 10.1093/brain/awl220. Epub 2006 Aug 18.
Spinal dural arteriovenous fistula (SDAVF) is a rare and enigmatic disease entity. The clinical features and structural changes have been recognized since 1926, and the pathophysiology and the essentials of treatment since 1974, but up to the present day it is unknown why these fistulas develop. The fistula between a radicular artery and the corresponding radicular vein within the dural root sleeve leads to congestion of the venous outflow of the spinal cord and eventually ischaemia. Patients, who are mostly middle-aged men, develop a progressive myelopathy, which at the early stages of the disease often mimics a polyradiculopathy or anterior horn cell disorder. By the time involvement of upper motoneurons or sacral segments makes the diagnosis of SDAVF inescapable, patients suffer from considerable neurological deficits. The diagnosis relies on MRI, which shows swelling of the spinal cord, with a centrally located hyperintense signal on T2-weighted images, and with hypointense 'flow void' phenomena dorsal to the cord, representing enlarged and tortuous veins. Catheter angiography is required to determine the exact location of the fistula as well as the angio-architecture, on which the mode of treatment depends. If the arterial feeder of the fistula is a tributary of the anterior spinal artery, embolization is not possible. After embolization recanalization may occur, but this is rarely seen after filling of the draining vein with glue. Alternatively, operation is a safe and permanent mode of treatment. No prognostic factors have been reliably established. Muscle strength and gait disturbances respond better to treatment than pain and symptoms related to damage of sacral segments. In any middle aged male patient with ascending motor or sensory deficits in the legs, SDAVF should be considered in order to prevent irreversible handicap.
脊髓硬脊膜动静脉瘘（SDAVF）是一种罕见且神秘的疾病实体。自1926年以来已认识到其临床特征和结构变化，自1974年以来已认识到其病理生理学和治疗要点，但直至今日仍不清楚这些瘘管为何会发生。硬脊膜神经根袖套内的根动脉与相应的根静脉之间的瘘管导致脊髓静脉回流受阻，最终导致缺血。患者多为中年男性，会出现进行性脊髓病，在疾病早期常类似多神经根病或前角细胞疾病。当出现上运动神经元或骶段受累而使SDAVF的诊断不可避免时，患者会出现相当严重的神经功能缺损。诊断依赖于MRI，其显示脊髓肿胀，在T2加权图像上有位于中央的高信号，且在脊髓背侧有低信号的“流空”现象，代表扩张和迂曲的静脉。需要进行导管血管造影以确定瘘管的确切位置以及血管结构，治疗方式取决于此。如果瘘管的动脉供血支是脊髓前动脉的分支，则无法进行栓塞。栓塞后可能会发生再通，但用胶水充盈引流静脉后很少见这种情况。另外，手术是一种安全且永久性的治疗方式。尚未可靠地确定预后因素。肌肉力量和步态障碍对治疗的反应比对疼痛和与骶段损伤相关的症状的反应更好。对于任何出现下肢进行性运动或感觉功能缺损的中年男性患者，都应考虑SDAVF，以防止不可逆转的残疾。
