BACKGROUND Focal cerebral arteriopathy (FCA), a major cause of childhood arterial ischemic stroke (AIS), can progress and lead to increased infarct size and/or recurrent stroke. Evaluating treatment options depends on the ability to quantify reliably the degree of stenosis in FCA. AIMS We validated the recently introduced FCA severity score (FCASS) in an independent cohort from the Swiss Neuro-Paediatric Stroke Registry (SNPSR). MATERIALS AND METHODS We included children with FCA who had MR or CT angiography and a Pediatric Stroke Outcome Measure (PSOM) at 6-months and 2-years post-stroke. A paediatric neuroradiologist applied the FCASS and the modified pediatric Alberta Stroke Program Early Computed Tomography Score (ASPECTS), a measure of infarct volume, to all available imaging. Two senior paediatric stroke neurologists and a neuroradiology fellow independently assigned FCASS scores to test interrater reliability. Pairwise correlations between FCASS, pedASPECTS, and PSOM were examined. RESULTS Thirty-two children [median (IQR) age = 5.9 (1.8, 9.6), 19 males] were included. The median maximum FCASS score at any time was 9 (IQR 6, 12; range 3, 16). Larger infarct volume scores correlated with both higher maximum FCASS scores and worse post-stroke outcomes, although we found no direct correlation between FCASS and outcomes. Stroke neurologists tended to assign lower FCASS scores than the neuroradiologist, but interrater reliability was predominantly good. CONCLUSIONS In this independent validation cohort, higher maximum FCASS correlated with greater infarct volume scores that also correlated with worse neurological outcomes. Scoring by non-imaging specialists seems to be valuable, although differences are present.

中文翻译：

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在瑞士队列中验证局灶性脑动脉病严重程度评分 (FCASS)：与梗死体积和结果的相关性

背景局灶性脑动脉病 (FCA) 是儿童动脉缺血性中风 (AIS) 的主要原因，可进展并导致梗塞面积增加和/或中风复发。评估治疗方案取决于可靠量化 FCA 狭窄程度的能力。目的 我们在来自瑞士神经儿科卒中登记处 (SNPSR) 的独立队列中验证了最近引入的 FCA 严重程度评分 (FCASS)。材料和方法 我们纳入了 FCA 儿童，他们在卒中后 6 个月和 2 年进行了 MR 或 CT 血管造影和小儿卒中结局测量 (PSOM)。一位儿科神经放射科医生将 FCASS 和改良的儿科阿尔伯塔中风计划早期计算机断层扫描评分 (ASPECTS)（梗塞体积的测量）应用于所有可用的成像。两名资深儿科中风神经学家和一名神经放射学研究员独立分配 FCASS 分数来测试评分者间的可靠性。检查了 FCASS、pedASPECTS 和 PSOM 之间的成对相关性。结果 包括 32 名儿童 [中位 (IQR) 年龄 = 5.9 (1.8, 9.6)，19 名男性]。任何时间的最大 FCASS 得分中位数为 9（IQR 6、12；范围 3、16）。较大的梗死体积分数与较高的最大 FCASS 分数和较差的卒中后结果相关，尽管我们发现 FCASS 与结果之间没有直接相关性。中风神经学家倾向于分配比神经放射学家更低的 FCASS 分数，但评分者之间的可靠性主要是好的。结论在这个独立的验证队列中，更高的最大 FCASS 与更大的梗死体积分数相关，后者也与更差的神经学结果相关。