Department of Pediatrics, Division of Neurology, The Hospital for Sick Children, Toronto, ON, Canada.
Department of Diagnostic Imaging, The Hospital for Sick Children, Toronto, ON, Canada.
Dev Med Child Neurol. 2021 Sep;63(9):1123-1126. doi: 10.1111/dmcn.14903. Epub 2021 May 6.
Mineralizing angiopathy is a unique, age-specific stroke syndrome characterized by basal ganglia infarction and lenticulostriate calcification after minor head injury in early childhood. There is limited understanding of the pathophysiology, course, and clinical outcome of this syndrome. We describe the clinical and radiographical phenotype of a single-center, consecutively enrolled cohort of children with mineralizing angiopathy from January 2002 to January 2020 and provide a comparative analysis to previously published literature. Fourteen children were identified. Previously unreported findings include: stroke onset in eight children older than 18 months; presence of basal ganglia hemorrhage in four; multifocal basal ganglia infarcts in three; presence of additional non-basal ganglia calcifications in three; and presence of thrombophilia in one. Seven children had moderate-to-severe neurological deficits. There was no symptomatic stroke recurrence (mean follow-up 3y 7mo, SD 1y 7mo). Our expanded phenotype highlights distinct characteristics of mineralizing angiopathy in children and has the potential to inform future research. What this paper adds Children with mineralizing angiopathy are often misdiagnosed as having a limb fracture despite normal x-rays. A magnetic resonance imaging-only approach may miss this entity. Non-contrast computed tomography, in addition to MRI is recommended to identify calcifications in idiopathic arterial ischemic stroke. Most children have moderate-to-severe neurological sequela.
矿化性血管病是一种独特的、年龄特异性的中风综合征，其特征为儿童早期轻微头部外伤后基底节梗死和纹状体钙化。对于这种综合征的病理生理学、病程和临床转归，人们的了解有限。我们描述了 2002 年 1 月至 2020 年 1 月期间在一家单中心连续入组的矿化性血管病患儿的临床和影像学表型，并与以前发表的文献进行了比较分析。共确定了 14 名儿童。以前未报道的发现包括：8 名儿童的中风发作年龄大于 18 个月；4 名存在基底节出血；3 名存在多发性基底节梗死；3 名存在其他非基底节钙化；1 名存在血栓形成倾向。7 名儿童有中度至重度神经功能缺损。无症状性中风复发（平均随访时间 3y7mo，SD1y7mo）。我们扩展的表型突出了儿童矿化性血管病的独特特征，并有潜力为未来的研究提供信息。本文的新发现矿化性血管病患儿常被误诊为骨折，尽管 X 线正常。单纯磁共振成像检查可能会漏诊该疾病。除了 MRI 之外，还建议进行非增强 CT，以识别特发性动脉缺血性中风中的钙化。大多数儿童有中度至重度神经后遗症。
