Interstitial spinal-cord oedema in syringomyelia associated with Chiari type 1 malformations
Interstitial spinal-cord oedema in syringomyelia associated with Chiari type 1 malformations
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DOI：
10.1136/jnnp.2007.133959
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发表时间：
2008-10-01
影响因子：
11
通讯作者：
Houkin, K.
中科院分区：
文献类型：
--
作者：
Akiyama, Y.;Koyanagi, I.;Houkin, K.
目的：基亚里1型畸形中脊髓压迫症的病理生理学尚未阐明。水肿样脊髓肿胀最近被报道在几种病理条件，包括基亚里1型畸形作为一个前syrinx状态。然而，脊髓空洞前状态在脊髓空洞症发展中的作用尚不清楚。方法：回顾性分析14例经枕大孔减压术治疗的基亚里1型畸形合并脊髓空洞症患者的术前、术后MRI表现。分析的重点是T1和T2加权图像和异常实质信号变化之间的空洞可视化的差异。男6例，女8例，年龄6 ~ 79岁。结果：12例患者的空洞较大且扩张，2例患者的空洞较小，局限于脊髓中央。T2加权像显示髓内异常信号区明显增大。9例患者在扩大的中央管周围或邻近脊髓空洞的后白色柱底部显示实质高信号区。这样的实质高信号区显着减少手术后的空洞减少，被认为是间质性oedema.Conclusions：从这项研究中，间质性水肿的脊髓通常伴随脊髓灰质炎与基亚里1型畸形。由于吸收机制紊乱导致的细胞外液蓄积可能在与基亚里1型畸形相关的脊髓空洞症的病理生理学中起重要作用。
Object: The pathophysiology of syringomyelia in Chiari type 1 malformations has not been clarified. Oedema-like spinal-cord swelling was recently reported in several pathological conditions, including Chiari type 1 malformations as a pre-syrinx state. However, the role of the pre-syrinx state in the development of syringomyelia is unknown. The purpose of this study is to investigate the parenchymal changes of the spinal cord in syringomyelia associated with Chiari type 1 malformations.Methods: Pre- and postoperative MRI findings in 14 patients who underwent foramen magnum decompression in our institute were reviewed. The analysis was focused on differences in visualisation of the syrinx between T1- and T2-weighted images and abnormal parenchymal signal changes. There were 6 men and 8 women, aged from 6 to 79 years. No patients showed hydrocephalus.Results: Twelve patients had large and expansive syrinx, whereas 2 patients showed small syrinx confined to the centre of the spinal cord. T2-weighted images displayed significantly larger intramedullary abnormal signal areas. Nine patients showed parenchymal hyperintensity areas around the enlarged central canal or base of the posterior white columns adjacent to the syringomyelic cavity. Such parenchymal hyperintensity areas markedly diminished with reduction of the syrinx after surgery and were considered to be interstitial oedema.Conclusions: From this study, the interstitial oedema of the spinal cord commonly accompanies syringomyelia with Chiari type 1 malformations. Accumulation of the extracellular fluid due to disturbed absorption mechanisms may play an important role in the pathophysiology of syringomyelia associated with Chiari type 1 malformations.