Characteristics of Moyamoya Syndrome in Sickle-Cell Disease by Magnetic Resonance Angiography: An Adult-Cohort Study
Characteristics of Moyamoya Syndrome in Sickle-Cell Disease by Magnetic Resonance Angiography: An Adult-Cohort Study
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DOI：
10.3389/fneur.2019.00015
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发表时间：
2019-01-22
影响因子：
3.4
通讯作者：
Bartolucci, Pablo
中科院分区：
文献类型：
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作者：
Kauv, Paul;Gaudre, Noemie;Bartolucci, Pablo
背景：镰状细胞病(SCD)可合并烟雾病综合征。脑磁共振血管成像(MRA)是一种无创诊断该综合征的方法，狭窄闭塞和烟雾血管(MMV)评分可用来评估其严重程度。以前对SCD烟雾综合征的研究并没有根据MRA评分来评估病情的严重程度。目的：使用这些MRA评分来评估SCD成人队列中烟雾综合征的特征。方法：对25例SCD合并烟雾综合征患者进行MRA检查，并结合3D飞行时间技术。我们从颈内动脉(ICA)狭窄程度(0-3)、大脑前动脉(ACA)(0-3)、大脑中动脉(MCA)(0-2)和大脑后动脉(PCA)(0-2)来评估每个半球(范围0-10)的狭窄程度评分。每个大脑半球的MMV评分(范围0~5)取决于5个MMV区域：(1)前交通动脉(2)基底节(3)ICA/MCA(4)后交通动脉/PCA(5)基底动脉。结果：8例(32%)患者出现单侧烟雾综合征。ICA狭窄22例(88%)，MCA 23例(92%)，ACA 9例(36%)，PCA 3例(12%)。MMV累及ACoA区10例(40%)，基底节区13例(52%)，PCoA/PCa 10例(40%)，MCA/ICA 7例(28%)，基底动脉1例(4%)。狭窄闭塞和MMV平均大脑半球评分分别为3.4/10(+/-1.42)和1.6/5(+/-0.71)。结论：单侧烟雾综合征多发，少见的PCa受累，中度狭窄和MMV评分似乎是SCD烟雾综合征的特征。在未来的研究中，可以收集MRA评分来评估这些患者的随访情况。
Background:Sickle cell disease (SCD) can be complicated by moyamoya syndrome. Brain magnetic resonance angiography (MRA) is a non-invasive method to diagnose this syndrome and, steno-occlusion and moyamoya vessels (MMV) scores have been proposed to evaluate its severity. Previous studies of SCD moyamoya syndrome did not evaluate the severity according to MRA scores. The objective was to assess the characteristics of moyamoya syndrome in an adult cohort of SCD using these MRA scores.Methods: Twenty-five SCD patients with moyamoya syndrome were included using MRA with 3D time of flight technique. We evaluate steno-occlusion score for each hemisphere (range 0-10) from: steno-occlusion severity of internal carotid (ICA) (0-3), anterior cerebral (ACA) (0-3), middle cerebral (MCA) (0-2), and posterior cerebral (PCA) (0-2) arteries. MMV score for each hemisphere (range 0-5) depended from 5 MMV areas: (1) anterior communicating artery (2) basal ganglia (3) ICA/MCA (4) posterior communicating artery/PCA (5) basilar artery.Results: Eight patients (32%) showed unilateral moyamoya syndrome. ICA steno-occlusion was involved in 22 patients (88%), MCA in 23 patients (92%), ACA in 9 patients (36%), and PCA in 3 patients (12%). MMV involved ACoA area in 10 patients (40%), basal ganglia in 13 patients (52%), PCoA/PCA in 10 patients (40%), MCA/ICA in 7 patients (28%), and BA in 1 patient (4%). Steno-occlusion and MMV mean hemisphere scores were 3.4/10 (+/- 1.42) and 1.6/5 (+/- 0.71), respectively.Conclusion: Frequent unilateral moyamoya syndrome, uncommon PCA involvement and, moderate steno-occlusion and MMV scores seem to be features of SCD moyamoya syndrome. In future studies, MRA scores could be collected to assess the follow-up in these patients.