Lenticulostriate arterial lumina are normal in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy: a high-field in vivo MRI study.
OBJECTIVE
Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary small vessel disease. Although postmortem studies have demonstrated mural thickening in leptomeningeal arteries and lenticulostriate perforating arteries, it is unclear whether this also leads to luminal narrowing. High-field MRI scanners enable in vivo imaging of the lumen of the lenticulostriate arteries. The aim of this study is to examine the luminal diameters of lenticulostriate arteries in living patients with CADASIL and to investigate whether luminal narrowing is correlated with the number of lacunar infarcts in the basal ganglia.


METHODS
Twenty-two NOTCH3 mutation carriers and 11 healthy control subjects were examined using high-resolution 3-dimensional time-of-flight MR angiography imaging on a 7-T MRI scanner. Scans were analyzed for the presence of focal stenotic segments. The total number, length, and total cross-sectional area of lenticulostriate arteries were measured and compared between mutation carriers and control subjects. These measurements were correlated with age, disease duration, and number of lacunar infarcts in the basal ganglia.


RESULTS
No stenotic segments were observed. No differences between mutation carriers and control subjects were found in total number of end branches (mutation carriers: mean, 14.6; control subjects: mean, 12.8), length of the lenticulostriate system, or total cross-sectional area of lenticulostriate artery lumina. Measurements of lenticulostriate artery lumina were not associated with lacunar infarct load in the basal ganglia area or with basal ganglia hyperintensities.


CONCLUSIONS
Three-dimensional time-of-flight MR angiographic on 7 T showed no differences in luminal diameters of lenticulostriate arteries between patients with CADASIL and control subjects.
Department of Radiology, Leiden University Medical Center, ZA Leiden, The Netherlands. m.k.liem@lumc.nl
Michael K
MK
Liem
Jeroen
J
van der Grond
Maarten J
MJ
Versluis
Joost
J
Haan
Andrew G
AG
Webb
Michel D
MD
Ferrari
Mark A
MA
van Buchem
Saskia A J
SA
Lesnik Oberstein
D000328:Adult; D000367:Age Factors; D000368:Aged; D019412:Anatomy, Cross-Sectional; D001479:Basal Ganglia / Q000473:pathology; D020144:Basal Ganglia Cerebrovascular Disease / Q000473:pathology*; D046589:CADASIL / Q000453:epidemiology / Q000473:pathology*; D002536:Cerebral Arteries / Q000473:pathology; D003251:Constriction, Pathologic; D005260:Female; D006579:Heterozygote; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009154:Mutation; D000071656:Receptor, Notch3; D051880:Receptors, Notch / Q000235:genetics
C499374:NOTCH3 protein, human; D000071656:Receptor, Notch3; D051880:Receptors, Notch