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Virtual Pediatric Hospital: Paediapaedia: Sturge-Weber Syndrome Paediapaedia: Neurological Diseases

Sturge-Weber Syndrome

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
A cutaneous vascular nevus is seen usually along the distribution of the trigeminal nerve. Symptoms consist of seizures, contralateral hemiparesis, hemiatrophy, glaucoma, mental retardation, and abnormal behavior.

Etiology/Pathophysiology:
Small, thin walled angiomatous vessels in the pia of the cerebral convexities cause pericapillary calcification of the underlying cortex and the ipsilateral cutaneous vascular nevus (port wine flame nevus).

Pathology:
Small thin walled angiomatous vessels in the pia of the cerebral convexities are associated with pericapillary calcification in the underlying cerebral cortex.

Imaging Findings:
Calcification is usually unilateral, asymmetric, and curvilinear and seen most frequently in the posterior parietal and occipital regions. Brain atrophy on the side of the angioma is seen with compensatory calvarial thickening, a small hemicranium, ventriculomegaly and wide subarachnoid spaces.

DDX:
Gyral Calcification

References:
See References Chapter.

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