Multimodality Imaging of Cortical and White Matter Abnormalities in Sturge-Weber Syndrome
C. Juhásza,b,
E.M. Haackec,
J. Huc,
Y. Xuanc,
M. Makkia,
M.E. Behena,b,
M. Maqboola,
O. Muzika,c,
D.C. Chugania,c and
H.T. Chugania,b,c
a Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, Detroit, Mich
b Department of Neurology, Children's Hospital of Michigan, Wayne State University, Detroit, Mich
c Department of Radiology, Children's Hospital of Michigan, Wayne State University, Detroit, Mich
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Fig 1. Images of a 8.1-year-old girl (patient 12) with Sturge-Weber syndrome affecting the left hemisphere. Postgadolinium T1-weighted MR images show an extensive left hemispheric angioma. SWI shows gyriform cortical signal intensity abnormalities in the left posterior region (arrowheads). A separate area of abnormal SWI is also seen in the left frontal region, located at the gray/white matter junction (thick arrows). Severe hypometabolism on PET is confined to the posterior region, matching well with the cortical SWI changes. In addition to cortical MR imaging abnormalities, abnormal transmedullary deep veins in white matter and periventricular veins are seen on both gadolinium-enhanced T1 and SWI images (thin arrows).
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Fig 2. Co-registered SWI and PET images of a 6-year-old girl (patient 9) with Sturge-Weber syndrome affecting the left hemisphere. SWI shows gyriform cortical signal intensity abnormalities in the left posterior region in atrophic cortex with severe (>20% decrease) hypometabolism on PET. In front of this area, deep veins are seen in the white matter, connecting mildly hypometabolic (10%–20% decrease) cortex to deep, periventricular veins.
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Fig 3. Co-registered MR imaging and PET images of a 1.9-year-old boy (patient 2) with Sturge-Weber syndrome and right hemispheric involvement. Contrast-enhanced T1-weighted MR images show a small posterior angioma as well as subtle enhancement in some transmedullary veins. FDG-PET shows no hypometabolism in the right hemisphere. In contrast, SWI demonstrates a number of prominent transmedullary veins in the right frontoparietal region (arrows on phase image); these are joining to a deep periventricular vein (arrowhead). These venous abnormalities are well visualized on the SWI phase images. Magnetic susceptibility effects are also seen in the region of the posterior angioma overlying atrophic right parietal cortex (thick arrow).
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Fig 4. T1-weighted and SWI MR imaging fused with PET images of a 2.8 year-old girl (patient 4) with Sturge-Weber syndrome affecting the right hemisphere. T1-weighted images show focal posterior parietal atrophy with leptomeningeal gadolinium enhancement and subtle enhancement in the white matter, consistent with transmedullary veins. The same deep veins are well visualized on SWI (thick arrows), apparently connected to enlarged periventricular veins (thin arrows on the enlarged fused SWI/PET image). FDG-PET shows severe hypometabolism in the area of focal atrophy, while the transmedullary veins reach adjacent cortical regions with mild or no hypometabolism. The SWI/PET fusion image shows well the configuration of multiple draining veins (arrows) originating from the edge of the hypometabolic area.
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Fig 5. Representative DTI fractional anisotropy (FA) and color vector map images of patient 9 with a left parietal angioma. FDG-PET showed severe hypometabolism in the parietal lobe (Fig 2), whereas SWI visualized deep frontocentral transmedullary veins (see affected area in the SWI panel on the right) apparently draining from mildly hypometabolic cortex. FA map demonstrates decreased FA values extending into the frontal lobe. On the color map, fiber directions are color-coded based on the main eigenvector (red, left-right, green, anteroposterior, blue, superior-inferior). Note that in the central white matter region encompassing the deep veins, red and dark purple-coded voxels dominate, indicating an increased number of voxels with transverse (left-right) as well as oblique direction of water diffusion as opposed to the right (normal) side containing more fibers with superior-inferior direction. Both FA and color maps show severe loss and disorganization of white matter in the left parietal area under the angioma (arrows).
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