AUTHOR=Zhang Chen , Li Wei , Li ShaoWu , Niu SongTao , Wang XinGao , Yu Xueying , Zhang ZaiQiang 

TITLE=Intracranial Large Artery Abnormalities and Association With Cerebral Small Vessel Disease in CADASIL

JOURNAL=Frontiers in Neurology

VOLUME=Volume 11 - 2020

YEAR=2020

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2020.00726

DOI=10.3389/fneur.2020.00726

ISSN=1664-2295

ABSTRACT=Background and objective: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited systemic arteriopathy, and the classic feature is small vessel lesion. Study on intracranial large arteries in CADASIL is not common. We aim to evaluate intracranial large arteries, describing the characteristics of large artery in CADASIL and association with cerebral small vessel associated lesions. 
Methods: 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 neuroimaging. 
Results: The study included 37 CADASIL patients. Twenty-eight of them (75.7%) had intracranial large artery abnormalities. Eighteen (48.6%) had congenital variations such as fenestration, vertebral artery (VA) hypoplasia and agenesis, 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. 
Conclusions: CADASIL patients can demonstrate various intracranial large artery abnormalities which might influence the development of microangiopathy. Assessment of great vessels seems essential in CADASIL.