Which MRI findings are characteristic of Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Other MRI findings include accelerated myelination around the LA [72] ; a large choroid plexus, the size of which correlates with the extent of the LA [73] ; and progressive sinovenous occlusion on MR venography. Of note, Benedikt et al reported pial angiomatosis with adjacent cortical atrophy on MRI in 4 patients in whom unenhanced MRI or CT scan was normal or showed only nonspecific findings. [74]

Juhasz et al reported that cerebral hemisphere white matter volume ipsilateral to the angioma was an independent predictor of IQ, and that loss of such white matter volume may play a significant role in cognitive impairment in children with SWS. [75]

Bernal and Altman reported abnormal activation patterns in the occipital areas on functional MRI in patients with SWS. [76]

Lin et al reported perfusion MRI findings compatible with impaired venous drainage in an early case of SWS with new-onset seizures. [77]

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