From the Department of Radiology, Seattle Children's and University of Washington (F.A.P.).
University Children's Hospital, Zurich, Switzerland (G.O.).
Stroke. 2020 Mar;51(3):853-859. doi: 10.1161/STROKEAHA.119.027917. Epub 2020 Jan 2.
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; <0.01) and arteriopathy severity (Focal Cerebral Arteriopathy Severity Score; rho=0.85; <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; <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.
背景与目的-局灶性大脑动脉炎-炎症型（FCA-i）是一种常见的儿童动脉缺血性脑卒中类型，其血管造影特征为大前循环动脉局灶性和单侧狭窄/不规则，推测其病因与炎症有关。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，可能改善 FCA-i 患者的诊断、指导治疗和预测结局。我们假设 FCA-i 患者的 VWE 更严重或更广泛，其动脉病变更严重、梗死更大、临床结局更差，且梗死进展/复发的风险增加。方法-我们在本院回顾性地确定了患有动脉缺血性脑卒中、被归类为 FCA-i 并接受了血管壁成像的儿科患者。回顾了临床数据，并将 1 年时的小儿脑卒中结局量表（Pediatric Stroke Outcome Measure）作为主要的临床终点。评估了神经影像学研究中的梗死大小、动脉病变严重程度（局灶性大脑动脉病变严重程度评分）和 VWE。结果-评估了 9 例有血管壁成像的 FCA-i 患者，1 年时的临床结局与初始梗死体积之间存在很强的相关性（Spearman 相关系数 rho=0.84；<0.01），与动脉病变严重程度（Focal Cerebral Arteriopathy Severity Score；rho=0.85；<0.01）之间也存在很强的相关性。与无进展/复发的患者相比，出现梗死进展/复发的患者在就诊时的局灶性大脑动脉病变严重程度评分更差（中位数[四分位数间距]；9.0[8.0-11.8]与 5.0[4.0-7.0]；<0.05）。相反，VWE 测量值与动脉病变严重程度、梗死大小、临床结局或梗死进展/复发风险无关。此外，并非所有 FCA-i 患者均有 VWE。结论-VWE 可能不是 FCA-i 的可靠诊断或评估生物标志物，需要进一步研究来评估血管壁成像在儿科动脉缺血性脑卒中和 FCA-i 中的应用。
