Oesch Gabriela, Perez Francisco A, Wainwright Mark S, Shaw Dennis W W, Amlie-Lefond Catherine
Department of Pediatric Neurology, University Children's Hospital, Zurich, Switzerland (G.O.).
Department of Radiology, Seattle Children's and University of Washington (F.A.P., D.W.W.S.).
Stroke. 2021 Jul;52(7):2258-2265. doi: 10.1161/STROKEAHA.120.031880. Epub 2021 May 27.
Focal cerebral arteriopathy (FCA) of childhood with unilateral stenosis of the anterior circulation is reported to account for up to one-quarter of childhood arterial ischemic stroke, with stroke recurrence in 25% of cases. Limited knowledge regarding pathophysiology and outcome results in inconsistent treatment of FCA.
Children with arterial ischemic stroke due to FCA between January 1, 2009, and January 1, 2019, were retrospectively identified at our institution which serves the US Pacific Northwest region. Electronic health record data, including neuroimaging studies, were reviewed, and the Pediatric Stroke Outcome Measure at 1 year was determined as the primary clinical end point.
Fifteen children were diagnosed with FCA, accounting for 19% of children with cerebral arteriopathies (n=77). Among children with FCA, the median age at the time of stroke was 6.8 years (Q1–Q3, 1.9–14.0 years). Four (20%) patients had worsening stroke, 3 of whom had concurrent infection. Three (20%) FCA cases were treated with steroids, one of whom had worsening stroke. Median Pediatric Stroke Outcome Measure at 1 year was 1.0 (Q1–Q3, 0.6–2.0). Variability in arteriopathy severity was observed within many patients. Patients with more severe arteriopathy using the Focal Cerebral Arteriopathy Severity Score had larger strokes and were more likely to have worsening stroke. The most common long-term neurological deficit was hemiparesis, which was present in 11 (73%) patients and associated with middle cerebral artery arteriopathy and infarcts.
FCA may be less common than previously reported. Neuroimaging in FCA can help identify patients at greater risk for worsening stroke.
据报道，儿童局灶性脑动脉病变（FCA）伴前循环单侧狭窄占儿童动脉缺血性卒中的四分之一，25%的病例会复发卒中。由于对其病理生理学和预后的了解有限，导致FCA的治疗方法不一致。
对2009年1月1日至2019年1月1日期间在我们服务于美国太平洋西北地区的机构中因FCA导致动脉缺血性卒中的儿童进行回顾性识别。回顾电子健康记录数据，包括神经影像学研究，并将1年时的小儿卒中结局测量值确定为主要临床终点。
15名儿童被诊断为FCA，占脑动脉病变儿童的19%（n = 77）。在FCA儿童中，卒中时的中位年龄为6.8岁（第一四分位数 - 第三四分位数，1.9 - 14.0岁）。4名（20%）患者卒中病情恶化，其中3名并发感染。3例（20%）FCA病例接受了类固醇治疗，其中1例卒中病情恶化。1年时小儿卒中结局测量值的中位数为1.0（第一四分位数 - 第三四分位数，0.6 - 2.0）。在许多患者中观察到动脉病变严重程度的差异。使用局灶性脑动脉病变严重程度评分，动脉病变更严重的患者卒中面积更大，且更有可能出现卒中病情恶化。最常见的长期神经功能缺损是偏瘫，11名（73%）患者出现偏瘫，且与大脑中动脉病变和梗死有关。
FCA可能比先前报道的更为少见。FCA的神经影像学检查有助于识别卒中病情恶化风险更高的患者。
