Kauv Paul, Gaudré Noémie, Hodel Jérôme, Tuilier Titien, Habibi Anoosha, Oppenheim Catherine, Edjlali Myriam, Hervé Dominique, Calvet David, Bartolucci Pablo
Department of Neuroradiology, APHP, Hôpital Henri Mondor, UPEC, Créteil, France.
Unité des Maladies Génétiques du Globule Rouge, Sickle-Cell Referral Center, Internal Medicine, APHP, Hôpital Henri Mondor, UPEC, Créteil, France.
Front Neurol. 2019 Jan 22;10:15. doi: 10.3389/fneur.2019.00015. eCollection 2019.
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. 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. 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. 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.
镰状细胞病（SCD）可并发烟雾病综合征。脑磁共振血管造影（MRA）是诊断该综合征的一种非侵入性方法，并且已经提出了狭窄闭塞和烟雾血管（MMV）评分来评估其严重程度。先前关于SCD烟雾病综合征的研究未根据MRA评分评估严重程度。目的是使用这些MRA评分评估成年SCD队列中烟雾病综合征的特征。使用具有3D时间飞跃技术的MRA纳入了25例患有烟雾病综合征的SCD患者。我们根据以下各项评估每个半球的狭窄闭塞评分（范围0-10）：颈内动脉（ICA）（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）的狭窄闭塞严重程度。每个半球的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。8例患者（32%）表现为单侧烟雾病综合征。22例患者（88%）累及ICA狭窄闭塞，23例患者（92%）累及MCA，9例患者（36%）累及ACA，3例患者（12%）累及PCA。MMV累及前交通动脉区域10例患者（40%），基底节13例患者（52%），后交通动脉/PCA 10例患者（40%）, MCA/ICA 7例患者（28%），基底动脉1例患者（4%）。狭窄闭塞和MMV的平均半球评分分别为3.4/10（±1.42）和1.6/5（±0.71）。频繁出现的单侧烟雾病综合征、PCA受累不常见以及中度狭窄闭塞和MMV评分似乎是SCD烟雾病综合征的特征。在未来的研究中，可以收集MRA评分以评估这些患者的随访情况。
