Neurosciences and Mental Health Program, Stroke Imaging Laboratory for Children, The Hospital for Sick Children, Toronto, ON, Canada.
Department of Neuropsychology, The Hospital for Sick Children, Toronto, ON, Canada.
Transl Stroke Res. 2022 Oct;13(5):757-773. doi: 10.1007/s12975-022-01003-w. Epub 2022 Mar 25.
Moyamoya disease is a major arteriopathy characterised by progressive steno-occlusion of the arteries of the circle of Willis. Studies in adults with moyamoya suggest an association between abnormal fronto-parietal and white matter regional haemodynamics and cognitive impairments, even in the absence of focal infarction. However, these associations have not been investigated in children with moyamoya. We examined the relationship between regional haemodynamics and ratings of intellectual ability and executive function, using hypercapnic challenge blood oxygen level-dependent magnetic resonance imaging of cerebrovascular reactivity in a consecutive cohort of children with confirmed moyamoya. Thirty children were included in the final analysis (mean age: 12.55 ± 3.03 years, 17 females, 15 idiopathic moyamoya and 15 syndromic moyamoya). Frontal haemodynamics were abnormal in all regardless of stroke history and comorbidity, but occipital lobe haemodynamics were also abnormal in children with syndromic moyamoya. Executive function deficits were noted in both idiopathic and syndromic moyamoya, whereas intellectual ability was impaired in syndromic moyamoya, even in the absence of stroke. Analysis of the relative effect of regional abnormal haemodynamics on cognitive outcomes demonstrated that executive dysfunction was predominantly explained by right parietal and white matter haemodynamics independent of stroke and comorbidity, while posterior circulation haemodynamics predicted intellectual ability. These results suggest that parietal and posterior haemodynamics play a compensatory role in overcoming frontal vulnerability and cognitive impairment.
烟雾病是一种主要的动脉疾病，其特征是大脑Willis 环动脉进行性狭窄和闭塞。成人烟雾病研究表明，异常的额顶叶和白质区域血液动力学与认知障碍之间存在关联，即使没有局灶性梗死也是如此。然而，这些关联在儿童烟雾病中尚未得到研究。我们使用脑血管反应性的高碳酸血症挑战血氧水平依赖性磁共振成像，在一系列确诊的烟雾病患儿中检查了区域血液动力学与智力和执行功能评分之间的关系。最终分析纳入了 30 名儿童（平均年龄：12.55 ± 3.03 岁，17 名女性，15 名特发性烟雾病和 15 名综合征性烟雾病）。无论有无卒中史和合并症，所有儿童的额部血液动力学均异常，但综合征性烟雾病患儿的枕叶血液动力学也异常。特发性和综合征性烟雾病患儿均存在执行功能障碍，而综合征性烟雾病患儿的智力受损，即使无卒中也是如此。对区域异常血液动力学对认知结果的相对影响的分析表明，执行功能障碍主要由右侧顶叶和白质血液动力学解释，与卒中和合并症无关，而后循环血液动力学预测智力。这些结果表明，顶叶和后循环血液动力学在克服额叶脆弱性和认知障碍方面起着代偿作用。
