Sekula Raymond F, Arnone Gregory D, Crocker Christine, Aziz Khaled M, Alperin Noam
Department of Neurological Surgery, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Neurol Res. 2011 Apr;33(3):232-9. doi: 10.1179/016164111X12962202723724.
The pathogeneses of Chiari malformation type I and syringomyelia are incompletely understood. In this article, the authors attempt to review the current theories on the pathogeneses of Chiari I malformation and syringomyelia.
A literature review for articles pertaining to Chiari I malformation or syringomyelia before August 2010 was conducted; in addition, the author's own experience in treating Chiari I malformation and syringomyelia is included.
Chiari I malformation has been defined radiographically as cerebellar tonsillar herniation or ectopia 5 mm or greater below the foramen magnum. By this narrow definition, Chiari I malformation (i.e. cerebellar tonsillar herniation or ectopia 5 mm or greater below the foramen magnum) likely encompasses a heterogeneous grouping of disorders caused by different mechanisms. Molecular and genetic studies have been helpful in furthering our understanding of Chiari I malformation.
A review of the pathogeneses of Chiari I malformation and syringomyelia is reported.
I型Chiari畸形和脊髓空洞症的发病机制尚未完全明确。在本文中，作者试图对目前关于I型Chiari畸形和脊髓空洞症发病机制的理论进行综述。
对2010年8月之前有关I型Chiari畸形或脊髓空洞症的文章进行文献综述；此外，还纳入了作者自身治疗I型Chiari畸形和脊髓空洞症的经验。
I型Chiari畸形在影像学上被定义为小脑扁桃体疝或枕大孔以下5毫米或更大程度的异位。按照这个狭义的定义，I型Chiari畸形（即小脑扁桃体疝或枕大孔以下5毫米或更大程度的异位）可能涵盖了由不同机制引起的一组异质性疾病。分子和遗传学研究有助于加深我们对I型Chiari畸形的理解。
报道了对I型Chiari畸形和脊髓空洞症发病机制的综述。
