Changes over time in craniocerebral morphology and syringomyelia in cavalier King Charles spaniels with Chiari-like malformation
Changes over time in craniocerebral morphology and syringomyelia in cavalier King Charles spaniels with Chiari-like malformation
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DOI：
10.1186/1746-6148-8-215
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发表时间：
2012-11-08
影响因子：
2.6
通讯作者：
Volk, Holger A.
中科院分区：
文献类型：
--
作者：
Driver, Colin J.;De Risio, Luisa;Volk, Holger A.
背景：Chiari样畸形(CM)和脊髓空洞症是一种神经系统疾病综合体，在骑士查尔斯国王犬(CKCS)中发病率很高。这种疾病随时间的自然发展还没有被描述。本研究的目的是：1)确定脊髓空洞症是否随时间进展；2)确定先前与CM相关的颅脑形态特征是否为进行性的(包括尾侧颅窝体积、尾侧颅窝实质体积、脑室大小、枕大孔高度和小脑突出程度)。结果：脊髓空洞最大宽度、枕大孔高度、小脑突出长度和尾侧颅窝体积随时间增加。脑室和尾窝实质体积在不同扫描之间无显著差异。结论：本研究结果提示脊髓空洞症随时间进展。尾侧颅窝体积的增加可能与枕上骨的主动吸收有关，这是以前在成人CKCS的组织学标本中发现的。我们假设枕上骨的主动吸收是由于小脑的压力造成的。这些发现对于我们理解CKCS的CM和脊髓空洞症的发病机制和不同的自然临床进展具有重要的意义。
Background: Chiari-like malformation (CM) and syringomyelia is a neurological disease complex with high prevalence in cavalier King Charles spaniels (CKCS). The natural progression of this disease with time has not been described. The objectives of this study were to i) determine if syringomyelia progresses with time ii) determine if features of craniocrebral morphology previously associated with CM are progressive (including caudal cranial fossa volume, caudal cranial fossa parenchymal volume, ventricular dimensions, height of the foramen magnum and degree of cerebellar herniation). A retrospective morphometric analysis was undertaken in 12 CKCS with CM for which repeat magnetic resonance images were available without surgical intervention.Results: The maximal syrinx width, height of the foramen magnum, length of cerebellar herniation and caudal cranial fossa volume increased over time. Ventricular and caudal fossa parenchymal volumes were not significantly different between scans.Conclusions: The results of this study suggest that syringomyelia progresses with time. Increased caudal cranial fossa volume may be associated with active resorption of the supraoccipital bone, which has previously been found in histology specimens from adult CKCS. We hypothesise that active resorption of the supraoccipital bone occurs due to pressure from the cerebellum. These findings have important implications for our understanding of the pathogenesis and variable natural clinical progression of CM and syringomyelia in CKCS.