Arterial Spin-Labeling Perfusion Imaging in the Early Stage of Sturge-Weber Syndrome
AJNR Am J Neuroradiol. 2022-09-22; 43(10): 1516-1522
DOI: 10.3174/ajnr.A7643
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1. AJNR Am J Neuroradiol. 2022 Oct;43(10):1516-1522. doi: 10.3174/ajnr.A7643.
Epub 2022 Sep 22.
Arterial Spin-Labeling Perfusion Imaging in the Early Stage of Sturge-Weber
Syndrome.
Pouliquen G(1)(2), Fillon L(2), Dangouloff-Ros V(3)(2), Kuchenbuch M(4), Bar
C(4), Chemaly N(4), Levy R(3)(2), Roux CJ(3)(2), Saitovitch A(2), Boisgontier
J(2), Nabbout R(2)(4), Boddaert N(3)(2).
Author information:
(1)From the Department of Pediatric Radiology (G.P., V.D.-R., R.L., C.-J.R.,
N.B.) .
(2)Imagine Institute for Genetic Diseases (G.P., L.F., V.D.-R., R.L., C.-J.R.,
A.S., J.B., R.N., N.B.), L’Institut National de la Santé et de la Recherche
Médicale U1163, Paris, France.
(3)From the Department of Pediatric Radiology (G.P., V.D.-R., R.L., C.-J.R.,
N.B.).
(4)Centre de Reference Epilepsies Rares (M.K., C.B., N.C., R.N.), Department of
Pediatric Neurology, Necker Children’s Hospital, Assistance Publique-Hôpitaux de
Paris, Université de Paris, Paris, France.
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