Sturge-Weber Syndrome

Findings:
Multiple CT images show left parietooccipital gyral calcification, associated with focal cortical atrophy and enlargement of the ipsilateral choroid plexus.

Differential Diagnosis:
Sturge Weber syndrome, calcified infarct, celiac disease with folate deficiency

Discussion:
Sturge Weber syndrome is a rare angiomatous process of unknown inheritance that is included with the neurocutaneous syndromes. The syndrome is possibly related to persistent primordial sinusoidal vascular supply.
-clinical:
    -sz 90%
    -hemiplegia 30%
    -40-90% mental decline<2 years
    -glaucoma 30%
    -port wine stain in V1 distribution
-variants- facial/intracranial without eye, etc.
    I= facial nevus + pial angioma
    II= facial nevus
    III= pial angioma
 -trigeminal dermatome- all 3>>1+2>>other
-eye manifestations (33%):
    -bupthalmos-large globe
    -congenital glaucoma
    -choroidal angioma
    -episcleral telangiectasia
    -angiomas of EOM
-also assd with Klippel-Trenaunay Syndrome
-intracranial:
    -pial 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

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