Background

Prevalence and contribution of intracranial and extracranial arterial stenosis to stroke risk were assessed prospectively in children and young adults with sickle cell disease.

Methods

In this cross-sectional study, children and young adults (mean = 19.4 years) with sickle cell disease underwent neurological examination, brain MRI, and magnetic resonance angiography of the head and neck. Two neuroradiologists independently recorded infarcts and arterial stenosis. Clinical features and stroke outcomes were compared between participants with and without stenosis and between children and young adults. Logistic regression analysis assessed the association of variables of interest with overt stroke and silent cerebral infarct.

Results

Of 167 participants (79 children and 88 young adults), 20 (12.0%) had intracranial stenosis, all in the anterior circulation, and nine had concurrent extracranial stenosis. No participants had isolated extracranial stenosis. Participants with intracranial stenosis were more likely than those without stenosis to have an overt stroke (70% vs 5%, P < 0.001) or silent cerebral infarct (95% vs 35%, P < 0.001). Logistic regression analysis indicated that intracranial stenosis was strongly associated with overt stroke when compared with participants with silent cerebral infarct alone and strongly associated with silent cerebral infarct when compared with participants with normal brain MRI; male sex and age were also significant predictors of silent cerebral infarct.

Conclusions

Intracranial stenosis was strongly associated with both overt stroke and silent cerebral infarct; prevalence of intracranial stenosis was similar to prior estimates in sickle cell disease. Extracranial stenosis without concurrent intracranial stenosis did not occur and thus could not be evaluated as an independent risk factor for stroke.

中文翻译：

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颅内和颅外血管狭窄是镰状细胞病中风的危险因素

背景

在患有镰状细胞病的儿童和年轻人中，前瞻性地评估了颅内和颅外动脉狭窄对卒中风险的患病率和贡献。

方法

在这项横断面研究中，患有镰状细胞病的儿童和年轻人（平均 19.4 岁）接受了神经系统检查、脑部 MRI 和头颈部磁共振血管造影。两名神经放射科医生独立记录了梗塞和动脉狭窄。比较有狭窄和无狭窄的参与者以及儿童和年轻人之间的临床特征和中风结果。Logistic 回归分析评估了感兴趣的变量与明显中风和无症状脑梗塞的关联。

结果

在 167 名参与者（79 名儿童和 88 名年轻成人）中，20 名（12.0%）有颅内狭窄，均在前循环，9 名并发颅外狭窄。没有参与者有孤立的颅外狭窄。颅内狭窄的参与者比没有狭窄的参与者更可能发生明显的中风（70% 对 5%，P  < 0.001）或无症状脑梗塞（95% 对 35%，P  < 0.001）。Logistic回归分析表明，与单纯无症状脑梗塞的参与者相比，颅内狭窄与明显的卒中密切相关，与脑部MRI正常的参与者相比，与无症状脑梗塞密切相关；男性和年龄也是无症状脑梗塞的重要预测因素。

结论

颅内狭窄与明显中风和无症状脑梗塞密切相关；颅内狭窄的患病率与先前估计的镰状细胞病相似。没有并发颅内狭窄的颅外狭窄没有发生，因此不能作为卒中的独立危险因素进行评估。