PDF(Pubmed)
Abstract：
Moyamoya arteriopathy is a severe, progressive cerebral arteriopathy that places affected children at high risk for stroke. Moyamoya has been associated with a range of neuropsychological deficits in adults, but data on many cognitive domains remain limited in the pediatric population and little is known about the neuropsychological profile of children with syndromic moyamoya.
This is a single-center, retrospective cohort study of children with moyamoya arteriopathy followed at our center who underwent neuropsychological testing between 2003 and 2021. Test scores were extracted from neuropsychological reports. Medical records were reviewed with attention to individual neuropsychological test results, medical comorbidities, presence of infarct(s) on neuroimaging, and history of clinical ischemic stroke.
Of the 83 children with moyamoya followed at our center between 2003 and 2021, 13 had completed neuropsychological testing across multiple cognitive domains. Compared to age-based normative data, children in this sample had lower scores in overall intelligence (p = 0.003), global executive functioning (p = 0.005), and overall adaptive functioning (p = 0.015). There was no significant difference in overall intelligence between children with (n = 6) versus without (n = 7) a history of clinical stroke (p = 0.368), though children with any radiographic infarct scored lower in this domain (p = 0.032).
In our cohort, children with moyamoya demonstrated impaired intelligence and executive functioning, even in the absence of clinical stroke. Neuropsychological evaluation should be considered standard of care for all children with moyamoya, even those without a history of clinical stroke.
This is a single-center, retrospective cohort study of children with moyamoya arteriopathy followed at our center who underwent neuropsychological testing between 2003 and 2021. Test scores were extracted from neuropsychological reports. Medical records were reviewed with attention to individual neuropsychological test results, medical comorbidities, presence of infarct(s) on neuroimaging, and history of clinical ischemic stroke.
Of the 83 children with moyamoya followed at our center between 2003 and 2021, 13 had completed neuropsychological testing across multiple cognitive domains. Compared to age-based normative data, children in this sample had lower scores in overall intelligence (p = 0.003), global executive functioning (p = 0.005), and overall adaptive functioning (p = 0.015). There was no significant difference in overall intelligence between children with (n = 6) versus without (n = 7) a history of clinical stroke (p = 0.368), though children with any radiographic infarct scored lower in this domain (p = 0.032).
In our cohort, children with moyamoya demonstrated impaired intelligence and executive functioning, even in the absence of clinical stroke. Neuropsychological evaluation should be considered standard of care for all children with moyamoya, even those without a history of clinical stroke.
摘要：
背景：烟雾动脉病是一种严重的，进行性脑动脉病使受影响的儿童处于中风的高风险中。Moyamoya与成年人的一系列神经心理缺陷有关，但许多认知领域的数据在儿科人群中仍然有限，对患有烟雾综合征的儿童的神经心理学特征知之甚少。
方法：这是一个单中心，我们中心随访的2003~2021年接受神经心理学测试的烟雾动脉病患儿的回顾性队列研究.从神经心理学报告中提取测试分数。对医疗记录进行审查，并注意个人神经心理学测试结果，医疗合并症,神经影像学上是否存在梗死，和临床缺血性卒中病史。
结果：在2003年至2021年间在我们中心随访的83名烟雾病儿童中，有13名完成了跨多个认知领域的神经心理学测试。与基于年龄的规范数据相比，该样本中的儿童在总体智力上得分较低(p=0.003)，全球执行功能(p=0.005)，和整体自适应功能(p=0.015)。有临床卒中病史的儿童（n=6）与没有（n=7）的儿童（p=0.368）之间的总体智力没有显着差异，尽管有放射学梗死的儿童在该领域得分较低（p=0.032）。
结论：在我们的队列中，患有烟雾的儿童表现出智力和执行功能受损，即使没有临床中风。神经心理学评估应被视为所有烟雾患儿的护理标准，即使是那些没有临床中风史的人。
方法：这是一个单中心，我们中心随访的2003~2021年接受神经心理学测试的烟雾动脉病患儿的回顾性队列研究.从神经心理学报告中提取测试分数。对医疗记录进行审查，并注意个人神经心理学测试结果，医疗合并症,神经影像学上是否存在梗死，和临床缺血性卒中病史。
结果：在2003年至2021年间在我们中心随访的83名烟雾病儿童中，有13名完成了跨多个认知领域的神经心理学测试。与基于年龄的规范数据相比，该样本中的儿童在总体智力上得分较低(p=0.003)，全球执行功能(p=0.005)，和整体自适应功能(p=0.015)。有临床卒中病史的儿童（n=6）与没有（n=7）的儿童（p=0.368）之间的总体智力没有显着差异，尽管有放射学梗死的儿童在该领域得分较低（p=0.032）。
结论：在我们的队列中，患有烟雾的儿童表现出智力和执行功能受损，即使没有临床中风。神经心理学评估应被视为所有烟雾患儿的护理标准，即使是那些没有临床中风史的人。
