TY - JOUR T1 - Arterial Spin-Labeling Perfusion Imaging in the Early Stage of Sturge-Weber Syndrome JF - American Journal of Neuroradiology JO - Am. J. Neuroradiol. SP - 1516 LP - 1522 DO - 10.3174/ajnr.A7643 VL - 43 IS - 10 AU - G. Pouliquen AU - L. Fillon AU - V. Dangouloff-Ros AU - M. Kuchenbuch AU - C. Bar AU - N. Chemaly AU - R. Levy AU - C.-J. Roux AU - A. Saitovitch AU - J. Boisgontier AU - R. Nabbout AU - N. Boddaert Y1 - 2022/10/01 UR - http://www.ajnr.org/content/43/10/1516.abstract N2 - 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 ER -