Legacy and current understanding of the often-misunderstood Foix-Alajouanine syndrome. Historical vignette.
Legacy and current understanding of the often-misunderstood Foix-Alajouanine syndrome. Historical vignette.
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对经常被误解的 Foix-Alajouanine 综合征的传统和当前理解。
DOI：
10.3171/2007.11.17656
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发表时间：
2009
影响因子：
4.1
通讯作者：
M. Harrigan
中科院分区：
文献类型：
--
作者：
Andrew S. Ferrell;R. S. Tubbs;L. Acakpo;J. Deveikis;M. Harrigan
自1926年首次报道以来，Foix-Alajouanine综合征已成为一个众所周知的实体。传统的理解是，这种临床综合征是一种进行性脊髓静脉血栓形成相关的脊髓血管病变，导致坏死性脊髓病。然而，脊髓静脉血栓形成是非常罕见的，并不是任何常见的脊髓血管综合征的特征。翻译和审查原始42页的法语报告显示，2名年轻男子患有进行性和无情的脊髓病，最终导致死亡。病理分析显示原因不明的内膜间膜血管炎，包括血管壁增厚，但无管腔狭窄、脐带血管闭塞或血栓形成的证据。Foix和Alajouanine也排除了髓内动静脉畸形的存在。当时，还没有描述硬脑膜动静脉瘘（dAVF），因此没有专门寻找这种类型的病变。回想起来，这两名患者似乎都有I型dAVF导致的进行性脊髓病。在最初报告后的几十年里，许多作者将脊髓静脉血栓形成作为Foix-Alajouanine综合征的中心特征。在该综合征的临床表现中包含血栓形成不仅是不正确的，而且可能给人留下治疗无效的印象，从而可能阻止成功的手术或血管内治疗。
Foix-Alajouanine syndrome has become a well-known entity since its initial report in 1926. The traditional understanding of this clinical syndrome is as a progressive spinal cord venous thrombosis related to a spinal vascular lesion, resulting in necrotic myelopathy. However, spinal venous thrombosis is extremely rare and not a feature of any common spinal vascular syndrome. A translation and review of the original 42-page French report revealed 2 young men who had presented with progressive and unrelenting myelopathy ultimately leading to their deaths. Pathological analysis demonstrated endomesovasculitis of unknown origin, including vessel wall thickening without evidence of luminal narrowing, obliteration of cord vessels, or thrombosis. Foix and Alajouanine also excluded the presence of intramedullary arteriovenous malformations. At the time, dural arteriovenous fistulas (dAVFs) had not been described, and therefore this type of lesion was not specifically sought. In retrospect, it seems possible that both patients had progressive myelopathy due to Type I dAVFs. In the decades since that original report, numerous authors have included spinal cord venous thrombosis as a central feature of Foix-Alajouanine syndrome. The inclusion of thrombosis in the clinical picture of this syndrome is not only incorrect but may leave one with the impression of therapeutic futility, thus possibly preventing successful surgical or endovascular therapy.
颈脊髓静脉充血性脊髓病：尸检病例显示临床病程快速进展
DOI：
--
发表时间：
--
期刊：
Neuropathology (In press)
影响因子：
--
作者：
Kimura A;Tan CF;Wakida K;Saio M;Hozumi I;Inuzuka T;Takahashi H
通讯作者：
Takahashi H