Background and Purpose—Focal cerebral arteriopathy-inflammatory type (FCA-i) is a common cause of pediatric arterial ischemic stroke characterized angiographically by unifocal and unilateral stenosis/irregularity of the large anterior circulation arteries with a presumed inflammatory cause. Arterial vessel wall enhancement (VWE) on vessel wall magnetic resonance imaging is a potential biomarker of inflammation that may improve diagnosis, guide treatment, and predict outcomes in patients with FCA-i. We hypothesized that patients with FCA-i with more severe or extensive VWE would have worse arteriopathy, larger infarcts, worse clinical outcome, and increased risk for infarct progression/recurrence.Methods—Pediatric patients with arterial ischemic stroke, classified as FCA-i, and who underwent vessel wall imaging were retrospectively identified at our institution. Clinical data were reviewed and the Pediatric Stroke Outcome Measure at 1 year was determined as the primary clinical end point. Neuroimaging studies were assessed for infarct size, arteriopathy severity (Focal Cerebral Arteriopathy Severity Score), and VWE.Results—Nine cases of FCA-i with vessel wall imaging were evaluated, and there was a strong correlation between clinical outcome at 1-year with initial infarct volume (Spearman correlation coefficient rho=0.84; P<0.01) and arteriopathy severity (Focal Cerebral Arteriopathy Severity Score; rho=0.85; P<0.01). Patients with infarct progression/recurrence had worse Focal Cerebral Arteriopathy Severity Score at presentation compared with those without progression/recurrence (median [IQR]; 9.0 [8.0–11.8] and 5.0 [4.0–7.0], respectively; P<0.05). On the contrary, measures of VWE were not correlated with arteriopathy severity, infarct size, clinical outcome, or risk of infarct progression/recurrence. Moreover, not all patients with FCA-i demonstrated VWE.Conclusions—VWE may not be a reliable biomarker for the diagnosis or assessment of FCA-i, and future work is needed to assess the utility of vessel wall imaging in pediatric arterial ischemic stroke and FCA-i.

中文翻译：

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小儿局灶性脑动脉病-炎症亚型的MRI血管壁增强和其他成像生物标记物。

背景与目的-局灶性脑动脉炎-炎症型（FCA-i）是小儿动脉缺血性卒中的常见病因，其血管造影特点是大动脉前循环动脉的单灶性和单侧狭窄/不规则，并推测为炎症性原因。血管壁磁共振成像上的动脉血管壁增强（VWE）是炎症的潜在生物标志物，可改善FCA-i患者的诊断，指导治疗并预测结局。我们假设患有FCA-i且VWE更为严重或广泛的患者会出现更严重的动脉病变，更大的梗塞，更糟糕的临床预后以及梗塞进展/复发的风险增加。方法-小儿患有动脉缺血性卒中的患者，分类为FCA-i，在我们的机构中​​回顾性地确定了谁进行了血管壁成像。回顾了临床数据，并确定了1年的小儿卒中预后指标作为主要的临床终点。评估了神经影像学检查的梗死面积，动脉病变严重程度（局灶性脑动脉病变严重程度评分）和VWE。结果-评价了9例接受血管壁成像的FCA-i病例，并且1年的临床结局与2年的临床结局之间存在很强的相关性。初始梗塞体积（Spearman相关系数rho = 0.84;P <0.01）和动脉病变严重程度（局灶性脑动脉病变严重程度评分； rho = 0.85；P <0.01）。与无进展/复发的患者相比，有梗死进展/复发的患者在病灶时局灶性脑动脉病严重程度评分较差（中位数[IQR]；分别为9.0 [8.0-11.8]和5.0 [4.0-7.0]；P <0.05）。相反，VWE的测量值与动脉病变的严重程度，梗死面积，临床结局或梗塞进展/复发的风险无关。此外，并非所有的FCA-i患者都表现出VWE。结论— VWE可能不是诊断或评估FCA-i的可靠生物标志物，需要进一步的工作来评估血管壁成像在小儿动脉缺血性卒中和卒中中的作用。 FCA-i。