Zhang Chen, Li Wei, Li ShaoWu, Niu SongTao, Wang XinGao, Yu Xueying, Zhang ZaiQiang
Department of Neurology, China National Clinical Research Center for Neurological Diseases, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Department of Neuroimaging, Beijing Neurosurgical Institute, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Front Neurol. 2020 Aug 18;11:726. doi: 10.3389/fneur.2020.00726. eCollection 2020.
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited systemic arteriopathy, the classic feature of which is small vessel lesions. Studies on intracranial large arteries in CADASIL are not common. We aim to evaluate intracranial large arteries, describing the characteristics of large arteries in CADASIL and their association with cerebral small vessel associated lesions. Consecutive CADASIL patients from a single-center prospective cohort were analyzed. Brain magnetic resonance imaging and magnetic resonance angiography were performed to assess the intracranial large arteries and cerebral small vessels associated lesions' neuroimaging. The study included 37 CADASIL patients. Of the patients, 28 of them (75.7%) had intracranial large artery abnormalities. Eighteen (48.6%) had congenital variations such as fenestration, vertebral artery (VA) hypoplasia and agenesis, or common trunk and fetus posterior cerebral artery. Seventeen (45.9%) had acquired anomalies such as arterial stenosis, prolongation, or tortuosity (seven of them had both congenital and acquired anomalies). CADASIL patients with anterior circulation middle cerebral artery (MCA) or internal cerebral artery (ICA) severe stenosis were more likely to have ipsilateral asymmetric white matter hyper-density (WMH) distribution. Patients with posterior circulation VA hypoplasia had a higher prevalence of posterior subcortical zone dominant WMH distribution. CADASIL patients can demonstrate various intracranial large artery abnormalities which might influence the development of microangiopathy. Assessment of great vessels seems essential in CADASIL.
伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病（CADASIL）是一种遗传性全身性动脉病，其典型特征是小血管病变。关于CADASIL患者颅内大动脉的研究并不常见。我们旨在评估颅内大动脉，描述CADASIL患者大动脉的特征及其与脑小血管相关病变的关联。对来自单中心前瞻性队列的连续性CADASIL患者进行了分析。进行脑磁共振成像和磁共振血管造影以评估颅内大动脉和脑小血管相关病变的神经影像学表现。该研究纳入了37例CADASIL患者。其中，28例（75.7%）存在颅内大动脉异常。18例（48.6%）有先天性变异，如开窗、椎动脉（VA）发育不全或缺如、共同干以及胎儿型大脑后动脉。17例（45.9%）有后天性异常，如动脉狭窄、延长或迂曲（其中7例既有先天性又有后天性异常）。前循环大脑中动脉（MCA）或大脑内动脉（ICA）严重狭窄的CADASIL患者同侧白质高密度（WMH）分布不对称的可能性更大。后循环VA发育不全的患者皮质下后区为主的WMH分布患病率更高。CADASIL患者可表现出各种颅内大动脉异常，这可能会影响微血管病的发展。在CADASIL中评估大血管似乎至关重要。
