Akiyama Y, Koyanagi I, Yoshifuji K, Murakami T, Baba T, Minamida Y, Nonaka T, Houkin K
Department of Neurosurgery, Sapporo Medical University School of Medicine, Sapporo, Japan.
J Neurol Neurosurg Psychiatry. 2008 Oct;79(10):1153-8. doi: 10.1136/jnnp.2007.133959. Epub 2008 Apr 10.
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.
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.
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.
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.
Chiari I型畸形中脊髓空洞症的病理生理学尚未阐明。最近在几种病理情况下报道了类似水肿的脊髓肿胀,包括Chiari I型畸形作为空洞前状态。然而,空洞前状态在脊髓空洞症发展中的作用尚不清楚。本研究的目的是调查与Chiari I型畸形相关的脊髓空洞症中脊髓实质的变化。
回顾了我院14例行枕骨大孔减压术患者的术前和术后MRI表现。分析重点在于T1加权和T2加权图像上脊髓空洞显示的差异以及实质信号异常变化。患者共14例,男性6例,女性8例,年龄6至79岁。所有患者均无脑积水。
12例患者有大的扩展性脊髓空洞,而2例患者的脊髓空洞较小,局限于脊髓中央。T2加权图像显示髓内异常信号区域明显更大。9例患者在扩大的中央管周围或与脊髓空洞腔相邻的后白质柱基部出现实质高信号区。术后随着脊髓空洞缩小,这些实质高信号区明显减小,被认为是间质性水肿。
本研究表明,脊髓间质性水肿常伴随Chiari I型畸形相关的脊髓空洞症。吸收机制紊乱导致的细胞外液积聚可能在Chiari I型畸形相关脊髓空洞症的病理生理学中起重要作用。