Criscuolo G R, Oldfield E H, Doppman J L
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, Bethesda, Maryland.
J Neurosurg. 1989 Mar;70(3):354-9. doi: 10.3171/jns.1989.70.3.0354.
Acute or subacute neurological deterioration without evidence of hemorrhage in a patient with a spinal arteriovenous (AV) malformation has been referred to as "Foix-Alajouanine syndrome." This clinical entity has been considered to be the result of progressive vascular thrombosis resulting in a necrotic myelopathy; it has therefore been thought to be largely irreversible and hence untreatable. The authors report five patients with dural AV fistulas who presented in this manner, and who improved substantially after embolic and surgical therapy. The outcome of these patients indicates that acute and subacute progression of myelopathy in cases of spinal dural AV fistulas may be caused by venous congestion and not necessarily by thrombosis. Therefore, a clinical diagnosis of Foix-Alajouanine syndrome is of little practical use, as spinal cord dysfunction from venous congestion is a potentially reversible process whereas thrombotic infarction is not. This diagnosis may result in suboptimal management. The recognition of nonhemorrhagic acute or subacute myelopathy as a complication of a spinal dural AV fistula is important since what appears to be irreversible cord injury is often treatable by standard surgical techniques.
脊髓动静脉（AV）畸形患者出现无出血证据的急性或亚急性神经功能恶化，被称为“福-阿二氏综合征”。这种临床病症被认为是进行性血管血栓形成导致坏死性脊髓病的结果；因此，人们一直认为它在很大程度上是不可逆的，因而无法治疗。作者报告了5例以这种方式就诊的硬脊膜动静脉瘘患者，他们在接受栓塞和手术治疗后有显著改善。这些患者的治疗结果表明，脊髓硬脊膜动静脉瘘病例中脊髓病的急性和亚急性进展可能是由静脉充血引起的，而不一定是由血栓形成引起的。因此，福-阿二氏综合征的临床诊断几乎没有实际用途，因为静脉充血导致的脊髓功能障碍是一个潜在可逆的过程，而血栓形成性梗死则不是。这种诊断可能导致治疗效果欠佳。认识到非出血性急性或亚急性脊髓病是脊髓硬脊膜动静脉瘘的一种并发症很重要，因为看似不可逆的脊髓损伤通常可以通过标准手术技术进行治疗。
