Department of Diagnostic Radiology, School of Medicine, Tohoku University, Sendai, Japan.
AJNR Am J Neuroradiol. 2011 Jan;32(1):192-8. doi: 10.3174/ajnr.A2216. Epub 2010 Aug 26.
At diagnosis, the primary clinical manifestations of pediatric Moyamoya disease are TIA or CSs. CSs are reported to be more prevalent in younger than in older children. We sought to determine whether age-related differences in clinical manifestations are associated with age-related angiographic differences.
We divided 78 patients diagnosed with Moyamoya disease before 16 years of age into four 4-year age groups and examined the relationships between age at diagnosis and clinical manifestations and angiographic and MR imaging findings.
Among the 4 diagnostic age groups, in those younger than 4 years of age, the prevalence of CSs and of infarctions on MR images was highest, and along with severity of steno-occlusive lesions of the PCA, the prevalence was significantly higher than that in the next diagnostic age group (4-7 years), though the severity of steno-occlusive lesions in the ICA and the degree of transdural collaterals did not differ significantly. The prevalence of CSs and infarctions did not differ significantly in the 3 oldest diagnostic age groups, whereas ICA and PCA lesions and transdural collaterals correlated positively with diagnostic age.
The high prevalence of CSs and infarctions in patients diagnosed before 4 years of age is associated with advanced steno-occlusive lesions of the PCA. In patients 4 years of age and older at diagnosis, transdural collaterals develop in parallel with advancement of ICA and PCA lesions, which may contribute to the nearly constant prevalence of CSs.
在儿科烟雾病的诊断时,主要的临床表现为短暂性脑缺血发作或中风。据报道,中风在年龄较小的患儿中更为常见。我们试图确定临床表现与年龄相关的血管造影差异之间是否存在与年龄相关的差异。
我们将 78 例在 16 岁以下被诊断为烟雾病的患者分为四个 4 岁年龄组,并检查了诊断年龄与临床表现、血管造影和磁共振成像发现之间的关系。
在这四个诊断年龄组中,年龄小于 4 岁的患者中,中风和磁共振图像上梗死的发生率最高,而且与大脑后动脉(PCA)狭窄闭塞病变的严重程度相关,其发生率明显高于下一个诊断年龄组(4-7 岁),尽管颈内动脉(ICA)和硬脑膜侧支循环的狭窄闭塞程度无显著差异。在 3 个最年长的诊断年龄组中,中风和梗死的发生率没有显著差异,而 ICA 和 PCA 病变以及硬脑膜侧支循环与诊断年龄呈正相关。
在 4 岁以下被诊断的患者中,中风和梗死的高发生率与 PCA 的严重狭窄闭塞病变有关。在诊断时年龄为 4 岁及以上的患者中,硬脑膜侧支循环与 ICA 和 PCA 病变的进展平行发展,这可能导致中风的发生率几乎保持不变。