PT  - JOURNAL ARTICLE
AU  - Pouliquen, G.
AU  - Fillon, L.
AU  - Dangouloff-Ros, V.
AU  - Kuchenbuch, M.
AU  - Bar, C.
AU  - Chemaly, N.
AU  - Levy, R.
AU  - Roux, C.-J.
AU  - Saitovitch, A.
AU  - Boisgontier, J.
AU  - Nabbout, R.
AU  - Boddaert, N.
TI  - Arterial Spin-Labeling Perfusion Imaging in the Early Stage of Sturge-Weber Syndrome
AID  - 10.3174/ajnr.A7643
DP  - 2022 Sep 22
TA  - American Journal of Neuroradiology
4099  - http://www.ajnr.org/content/early/2022/09/22/ajnr.A7643.short
4100  - http://www.ajnr.org/content/early/2022/09/22/ajnr.A7643.full
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