From the Departments of Pediatrics (R.S.J., C.B., S.D.), Vanderbilt University Medical Center, Nashville, TN; Neurology (A.L.F., S.F., R.C.), Washington University, St. Louis, MO; Neurology (M.J.D.); Radiology (L.T.D., S.P.); Vanderbilt-Meharry Center of Excellence in Sickle Cell Disease (M.R.D.); Medicine (A.A.K.), Vanderbilt University Medical Center, Nashville, TN; Rodeghier Consulting (M.R.), Chicago, IL; Pediatrics, Neurology and Radiology (L.C.J.), Vanderbilt University Medical Center, Nashville, TN.
Neurology. 2024 May;102(10):e209247. doi: 10.1212/WNL.0000000000209247. Epub 2024 Apr 29.
Previously we demonstrated that 90% of infarcts in children with sickle cell anemia occur in the border zone regions of cerebral blood flow (CBF). We tested the hypothesis that adults with sickle cell disease (SCD) have silent cerebral infarcts (SCIs) in the border zone regions, with a secondary hypothesis that older age and traditional stroke risk factors would be associated with infarct occurrence in regions outside the border zones.
Adults with SCD 18-50 years of age were enrolled in a cross-sectional study at 2 centers and completed a 3T brain MRI. Participants with a history of overt stroke were excluded. Infarct masks were manually delineated on T2-fluid-attenuated inversion-recovery MRI and registered to the Montreal Neurological Institute 152 brain atlas to generate an infarct heatmap. Border zone regions between anterior, middle, and posterior cerebral arteries (ACA, MCA, and PCA) were quantified using the Digital 3D Brain MRI Arterial Territories Atlas, and logistic regression was applied to identify relationships between infarct distribution, demographics, and stroke risk factors.
Of 113 participants with SCD (median age 26.1 years, interquartile range [IQR] 21.6-31.4 years, 51% male), 56 (49.6%) had SCIs. Participants had a median of 5.5 infarcts (IQR 3.2-13.8). Analysis of infarct distribution showed that 350 of 644 infarcts (54.3%) were in 4 border zones of CBF and 294 (45.6%) were in non-border zone territories. More than 90% of infarcts were in 3 regions: the non-border zone ACA and MCA territories and the ACA-MCA border zone. Logistic regression showed that older participants have an increased chance of infarcts in the MCA territory (odds ratio [OR] 1.08; 95% CI 1.03-1.13; = 0.001) and a decreased chance of infarcts in the ACA-MCA border zone (OR 0.94; 95% CI 0.90-0.97; < 0.001). The presence of at least 1 stroke risk factor did not predict SCI location in any model.
When compared with children with SCD, in adults with SCD, older age is associated with expanded zones of tissue infarction that stretch beyond the traditional border zones of CBF, with more than 45% of infarcts in non-border zone regions.
此前我们发现，镰状细胞贫血患儿的 90%梗死发生在脑血流（CBF）的边缘区。我们验证了这样一个假说，即镰状细胞病（SCD）成年患者在边缘区存在无症状性脑梗死（SCI），并有一个次要假说，即年龄较大和传统的卒中危险因素与边缘区以外的梗死发生相关。
在两个中心进行了一项横断面研究，纳入了年龄在 18-50 岁的 SCD 成年患者，并完成了 3T 脑 MRI 检查。排除有显性卒中病史的患者。在 T2 液体衰减反转恢复 MRI 上手动勾画梗死灶，并将其注册到蒙特利尔神经学研究所 152 脑图谱，以生成梗死热图。使用数字 3D 脑 MRI 动脉区域图谱量化前、中、后大脑动脉（ACA、MCA 和 PCA）之间的边缘区，并应用逻辑回归来确定梗死分布、人口统计学和卒中危险因素之间的关系。
在 113 名 SCD 患者（中位年龄 26.1 岁，四分位间距[IQR] 21.6-31.4 岁，51%为男性）中，56 名（49.6%）患者存在 SCI。患者的中位梗死数量为 5.5 个（IQR 3.2-13.8）。分析梗死分布显示，644 个梗死灶中有 350 个（54.3%）位于 4 个 CBF 边缘区，294 个（45.6%）位于非边缘区。超过 90%的梗死发生在 3 个区域：非边缘区 ACA 和 MCA 区以及 ACA-MCA 边缘区。逻辑回归显示，年龄较大的患者 MCA 区梗死的几率增加（比值比[OR] 1.08；95%置信区间[CI] 1.03-1.13； = 0.001），而 ACA-MCA 边缘区梗死的几率降低（OR 0.94；95%CI 0.90-0.97； < 0.001）。在任何模型中，至少存在 1 个卒中危险因素与 SCI 部位均无相关性。
与 SCD 患儿相比，SCD 成年患者中，年龄较大与超出传统 CBF 边缘区的组织梗死区扩张有关，超过 45%的梗死发生在非边缘区。
