Support Center for Advanced Neuroimaging, Institute of Diagnostic and Interventional Neuroradiology (N.S.), Inselspital, Bern University Hospital, University of Bern, Switzerland.
Pediatric Radiology, University of Basel Children's Hospital and University of Basel, Switzerland (N.S.).
Stroke. 2024 Apr;55(4):1006-1014. doi: 10.1161/STROKEAHA.123.043562. Epub 2024 Mar 6.
Inflammatory type focal cerebral arteriopathy (FCA-i) in the anterior circulation (AC) is well characterized, and the focal cerebral arteriopathy severity score (FCASS) reflects the severity of the disease. We identified cases of FCA-i in the posterior circulation (PC) and adapted the FCASS to describe these cases.
In this comparative cohort study, patients from the Swiss NeuroPaediatric Stroke Registry with ischemic stroke due to FCA-i between January 2000 and December 2018 were analyzed. A comparison between PC and AC cases regarding pediatric National Institutes of Health Stroke Scale score and pediatric stroke outcome measure and FCASS was performed. We estimated infarct size by the modified pediatric Alberta Stroke Program Early Computed Tomography Score in children with AC stroke and the adapted Bernese posterior diffusion-weighted imaging score in the PC.
Thirty-five children with a median age of 6.3 (interquartile range, 2.7-8.2 [95% CI, 0.9-15.6]; 20 male; 57.1%) years with FCA-i were identified. The total incidence rate was 0.15/100 000/year (95% CI, 0.11-0.21). Six had PC-FCA-i. Time to final FCASS was longer in the PC compared with AC; the evolution of FCASS did not differ. Initial pediatric National Institutes of Health Stroke Scale score was higher in children with FCA-i in the PC with a median of 10.0 (interquartile range, 5.75-21.0) compared with 4.5 (interquartile range, 2.0-8.0) in those with AC-FCA-i. Different from the anterior cases, PC infarct volume did not correlate with higher discharge, maximum, or final FCASS scores (Pearson correlation coefficient [r], 0.25, 0.35, and 0.54).
FCA-i also affects the PC. These cases should be included in future investigations into FCA-i. Although it did not correlate with clinical outcomes in our cohort, the modified FCASS may well serve as a marker for the evolution of the arteriopathy in posterior FCA-i.
炎性型前循环局灶性脑动脉病（FCA-i）的特征已得到充分描述，局灶性脑动脉病严重程度评分（FCASS）反映了疾病的严重程度。我们在本研究中识别了后循环（PC）中的 FCA-i 病例，并对 FCASS 进行了调整，以描述这些病例。
本研究为一项比较队列研究，纳入了 2000 年 1 月至 2018 年 12 月期间瑞士神经儿科卒中登记处中因 FCA-i 导致缺血性卒中的患者。比较了 PC 与 AC 病例之间的小儿国立卫生研究院卒中量表评分、小儿卒中结局测量和 FCASS。我们通过改良的小儿阿尔伯塔卒中项目早期计算机断层扫描评分估计 AC 卒中患儿的梗死灶大小，通过改良的伯尔尼后部弥散加权成像评分估计 PC 卒中患儿的梗死灶大小。
共识别出 35 例中位年龄为 6.3 岁（四分位距，2.7-8.2 岁；95%CI，0.9-15.6 岁；20 例男性；57.1%）的 FCA-i 患儿。总的发病率为 0.15/100000/年（95%CI，0.11-0.21）。其中 6 例为 PC-FCA-i。与 AC 相比，PC 患者的最终 FCASS 时间更长，而 FCASS 的演变没有差异。与 AC-FCA-i 患儿相比，PC-FCA-i 患儿的初始小儿国立卫生研究院卒中量表评分更高，中位数为 10.0（四分位距，5.75-21.0），而 AC-FCA-i 患儿的中位数为 4.5（四分位距，2.0-8.0）。与前循环病例不同的是，PC 梗死体积与较高的出院时、最大时和最终 FCASS 评分无相关性（Pearson 相关系数[r]，0.25、0.35 和 0.54）。
FCA-i 也可影响 PC。这些病例应纳入未来的 FCA-i 研究中。虽然在本队列中与临床结局无相关性，但改良的 FCASS 可能是反映 PC 后循环 FCA-i 病变演变的一个标志物。
