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Tuberous Sclerosis - Case 2

A 4 year-old girl presented with seizures and a delay in motor milestones.

Outline the Lesion

Tuberous Sclerosis (TS): T1-weighted with gadolinium axial MRI. Note the large, enhancing subependymal nodule on the right. This is a giant tuber in a patient with TS.

TS is a multisystemic genetic disorder with prominent CNS manifestations. Only a subset of patients display the classic triad of intractable seizures, mental retardation, and dermal angiofibromas. Several abnormalities may be present on brain imaging, including cortical tubers (hamartomas), bilateral and multiple subependymal nodules, and subependymal giant-cell astrocytomas that are most frequently found at the foramen of Monro.


Revised 11/29/06
Copyrighted 2006. David C Preston