Sturge Weber Syndrome

nRare neurocutaneous syndrome of unknown inheritance, may be related to persistent primordial sinusoidal vascular supply

nClinical

n-sz 90%
    -hemiplegia 30%
    -40-90% mental decline at <2 years of age
    -glaucoma 30%- also angiomas, telangiectasias, buphtalmos
    -port wine stain in V1 distribution

nMay be assd with Klippel Trenaunay syndrome- limb overgrowth, varicosities, nevi

nImaging

npial angioma with calcification, paucity of cortical veins, retrograde drainage to medullary veins
    -enlarged choroid plexus
    -most facial nevi overlie affected brain
    -chronic cerebral ischemia underlying pial angioma,
    -progressive cerebral calcification (unusual <2yrs)
    -may have cerebral hemiatrophy with calvarial thickening and enlarged sinuses
    -most common in parietooccipital

nVariants

nI- both facial nevus and pial angioma, II- nevus only, III- angioma only

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