RT Journal Article
SR Electronic
T1 Arterial Spin-Labeling Perfusion Imaging in the Early Stage of Sturge-Weber Syndrome
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
DO 10.3174/ajnr.A7643
A1 Pouliquen, G.
A1 Fillon, L.
A1 Dangouloff-Ros, V.
A1 Kuchenbuch, M.
A1 Bar, C.
A1 Chemaly, N.
A1 Levy, R.
A1 Roux, C.-J.
A1 Saitovitch, A.
A1 Boisgontier, J.
A1 Nabbout, R.
A1 Boddaert, N.
YR 2022
UL http://www.ajnr.org/content/early/2022/09/22/ajnr.A7643.abstract
AB BACKGROUND AND PURPOSE: Sturge-Weber syndrome is a rare congenital neuro-oculo-cutaneous disorder. Although the principal mechanism of Sturge-Weber syndrome is characterized by a leptomeningeal vascular malformation, few data regarding perfusion abnormalities of the brain parenchyma are available. Therefore, the aim of this study was to assess the diagnostic performance of arterial spin-labeling perfusion imaging in the early stage of Sturge-Weber syndrome before 1 year of age until 3.5 years of age. We hypothesized that a leptomeningeal vascular malformation has very early hypoperfusion compared with controls with healthy brains.MATERIALS AND METHODS: We compared the CBF using arterial spin-labeling perfusion imaging performed at 3T MR imaging in the brain parenchymal regions juxtaposing the leptomeningeal vascular malformation in patients with Sturge-Weber syndrome (n = 16; 3.5 years of age or younger) with the corresponding areas in age-matched controls with healthy brains (n = 58). The analysis was performed following two complementary methods: a whole-brain voxel-based analysis and a visual ROI analysis focused on brain territory of the leptomeningeal vascular malformation.RESULTS: Whole-brain voxel-based comparison revealed a significant unilateral decrease in CBF localized in the affected cortices of patients with Sturge-Weber syndrome (P < .001). CBF values within the ROIs in patients with Sturge-Weber syndrome were lower than those in controls (in the whole cohort: median, 25 mL/100g/min, versus 44 mL/100g/min; P < .001). This finding was also observed in the group younger than 1 year of age, emphasizing the high sensitivity of arterial spin-labeling in this age window in which the diagnosis is difficult.CONCLUSIONS: Arterial spin-labeling perfusion imaging in the early stage of Sturge-Weber syndrome can help to diagnose the disease by depicting a cortical hypoperfusion juxtaposing the leptomeningeal vascular malformation.ASLarterial spin-labelingAUCarea under the curveFWEfamily-wise errorIQRinterquartile rangeLVMleptomeningeal vascular malformationMNIMontreal Neurological InstitutePWBport-wine birthmarkSWSSturge-Weber syndrome