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Virtual Pediatric Hospital: Paediapaedia: Tuberous Sclerosis Paediapaedia: Neurological Diseases

Tuberous Sclerosis

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

Clinical Presentation:
Clinical triad is adenoma sebaceum, seizures, and mental retardation.

A hamartomatous proliferation of fibrous and connective tissue in the brain, skin, skeleton, kidneys, and heart.

The hamartomatous lesions in the brain consist of proliferating glial tissue and ganglion cells.

Imaging Findings:
The intracranial tubers are usually calcified and usually are in a periventricular / subependymal location but they can occasionally be found in the brain parenchyma. The tubers may undergo malignant degeneration into giant cell astrocytomas.


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